Hemolytic anemia according to ICD 10. D55-D59 Anemia due to enzyme disorders

D50- D53 anemia associated with eating:

D50-zalizodeficient;

D51 - vitamin B 12 - deficient;

D52-folate-deficient;

D53 - other anemia associated with eating.

D55- D59 - hemolytic anemia:

D55 - associated with enzymatic damage;

D56-thalasemy;

D57 - sickle-clitin;

D58 - more progressive hemolytic anemia;

D59-gostrі pridbanі hemolytic.

D60- D64- aplastic and other anemias:

D60 - pridban chervonoklitinna aplasia (erythroblastopenia);

D61 - more aplastic anemia;

D62 - acute aplastic anemia;

D63 - anemia of chronic illnesses;

D64 - more anemia.

Pathogenesis

The supply of tissues with acid to protect erythrocytes - a form of blood elements, so as not to avenge the nucleus, the main obligatory erythrocyte borrows hemoglobin - a protein that calls acid. Trivality of life of erythrocytes is close to 100 deb. When the concentration of hemoglobin is lower than 100-120 g/l, the delivery of sour to the nirk decreases, which is a stimulus for the production of erythropoietin by interstitial clitins, which leads to the proliferation of erythropoietic parietal cells of the cystic brain. For normal erythropoiesis, it is necessary:

healthy cystic brain

healthy drugs that produce enough erythropoietin

a sufficient amount of elements of substrates, the necessary hematopoiesis (used for saliva).

Damage to one of these minds leads to the development of anemia.

1. Scheme of the erythrocytes adoption. (T..R. Harrison).

clinical picture

Clinical manifestations of anemia are characterized by severity, rapid development, and sickness. In Zvichayna, the oxyghemoglobin fabrics of the fabrics of the deprivation of the loud part of the Kisnya, can be lichen, can be the abundant mechanism of the GB at 20-30g/l zbilshu, viddach to the tissues of the tissues of the blood, anemia is miserable in Vipa in Vyzhezkoye in the Vipa according

When the concentration of Hb is lower than 70-80g/l, there is stupefaction, a back with physical anxiety, heartbeat, and a pulsating head.

In sick people with a frail age with cardiovascular diseases, there is an increase in pain syndrome in the heart, an increasing sign of heart failure.

Gostra blood loss to cause a rapid decrease in the number of erythrocytes and BCC. It is necessary to reassess the state of hemodynamics. Pererozdil blood flow and spasm of the veins cannot compensate for the acute blood loss over 30%. So ill to lie down, pronounced orthostatic hypotension tachycardia. The intake of over 40% blood (2000 ml) can lead to shock, signs of this - tachypnea and tachycardia in calm, stupor, cold sticky fluid, decreased arterial pressure. Necessary urgent renewal of the BCC.

In case of chronic bleeding, the bcc begins to develop independently, compensatory increases in the bcc and cardiac dysfunction develop. As a result, there is an increase in the upper pulse, a high pulse, an increase in the pulse pressure, through the acceleration of the blood flow through the valve, a systolic murmur is heard during auscultation.

The blistering of the skin curves and mucous membranes becomes littered with a decrease in Hb concentration to 80-100 g/l. A sign of anemia can also be the appearance of a Zhovtyanitsa. In case of sickness, the attention and the state of the lymphatic system are affected, the spleen and liver are expanded, the ossalgia is revealed (pain when the brushes are beaten, especially the sternum), the attention is due to turn on the petechia, ecchymosis and other signs of impaired coagulability of blood flow.

Anemia severity levels(for equal Hb):

slight decrease in Hb 90-120 g/l

average Hb 70-90 g/l

cuff Hb<70 г/л

edge of the cuff Hb<40 г/л

Getting to the establishment of the diagnosis of anemia, it is necessary to confirm the following nutrition:

What are the signs of bleeding, why did it come out already?

What are the signs of superfluous hemolysis?

What are signs of strangulation of cystic cerebral hematopoiesis?

What are the signs of damage to the exchange of the hall?

What are the signs of vitamin B12 deficiency of folic acid?

Class III. Diseases of the blood, hematopoietic organs, and other damages that cause the immune mechanism (D50-D89)

Excludes: autoimmune ailment (systemic) NOS (M35.9), next to death, which is due to the perinatal period (P00-P96), worsening of pregnancy, later than the prenatal period (O00-O99), congenital anomalies, chromosomal deformity (Q0) - Q99), endocrine ailments, eating disorders and impaired speech exchange (E00-E90), ailments caused by a human immunodeficiency virus [VIL] (B20-B24), trauma, disruption and other consequences), influx of external causes (S00-T98 newborn (C00-D48), symptoms, signs and symptoms of normal, clinical and laboratory findings, not classified in other categories (R00-R99)

Tsey class to replace such blocks:
D50-D53 Anemia associated with eating
D55-D59 Hemolytic anemia
D60-D64 Aplastic and other anemias
D65-D69 Impaired pharynx, purpura and other hemorrhagic conditions
D70-D77 Other blood diseases and hematopoietic organs
D80-D89 Okremі damage, scho to catch the immune mechanism

Zirochkoy assigned the following categories:
D77 Other disorders of blood and hematopoietic organs in diseases classified in other headings

ANEMIA, RELATED TO LIFE (D50-D53)

D50 Hallodeficiency anemia

Inclusions: anemia
. sideropenic
. hypochromic
D50.0 Zalizodeficitna anemia is secondary through blood loss (chronic). Posthemorrhagic (chronic) anemia.
Excluded: acute posthemorrhagic anemia (D62); congenital anemia due to fetal hemorrhage (P61.3)
D50.1 Sideropenic dysphagia. Kelly Paterson Syndrome. Plummer Vinson Syndrome
D50.8 Other severe anemias
D50.9 Hallodeficiency anemia, unspecified

D51 Vitamin B12 deficiency anemia

Excludes: vitamin B12 deficiency (E53.8)

D51.0 Vitamin-B12 - deficiency anemia due to deficiency of the intrinsic factor.
Anemia:
. Addison
. Birmer
. pernicious (congenital)
Congenital deficiency of an internal factor
D51.1 Vitamin-B12-deficiency anemia due to vitamin B12 ingestion with proteinuria.
Imerslund (Gresbeck) syndrome. Megaloblastic spondylitis anemia
D51.2 Transcobalamin II deficiency
D51.3 Other vitamin-B12-deficiency anemia associated with eating. Vegetarian anemia
D51.8 Other vitamin B12 deficiency anemia
D51.9 Vitamin B12 deficiency anemia, unspecified

D52 Folic acid deficiency anemia

D52.0 Folic acid deficiency anemia associated with eating. Megaloblastic alimentary anemia
D52.1 Folic acid deficiency anemia is drug-induced. If necessary, identify the medical zasib
vikoristovuyut additional code of sound reasons (class XX)
D52.8 Other folic acid deficiency anemia
D52.9 Folic acid deficiency anemia, unspecified. Anemia due to insufficient intake of folic acid, NOS

D53 Other anemia associated with eating

Inclusions: megaloblastic anemia, which does not give rise to vitamins;
nom B12 or folates

D53.0 Anemia due to lack of proteins. Anemia due to lack of amino acids.
Orotaciduric anemia
Excluded: Lesch-Nychen syndrome (E79.1)
D53.1 Other megaloblastic anemias, which are not classified in other categories. Megaloblastic anemia NOS.
Excluded: ailment Di Guglielmo (C94.0)
D53.2 Anemia, intoxicated with scurvy.
Excluded: scurvy (E54)
D53.8 Other clarified anemia, related to eating.
Anemia associated with deficiency:
. midi
. molybdenum
. zinc
Included: insufficiency of eating without a riddle about
anemia, like this:
. midi deficiency (E61.0)
. molybdenum deficiency (E61.5)
. zinc deficiency (E60)
D53.9 Anemia due to eating, unspecified. Just chronic anemia.
Excl.: anemia NOS (D64.9)

HEMOLYTIC ANEMIA (D55-D59)

D55 Anemia due to enzyme disorders

Excludes: drug-induced enzyme deficiency anemia (D59.2)

D55.0 Anemia due to deficiency of glucose-6-phosphate dehydrogenase [G-6-PD]. Favism. G-6-PD-deficiency anemia
D55.1 Anemia due to other impairment of glutathione metabolism.
Anemia due to enzyme deficiencies (because of G-6-PD) associated with hexose monophosphate [HMF]
bypass of the metabolic pathway. Hemolytic non-spherocytic anemia (spadkov) type 1
D55.2 Anemia due to disruption of glycolytic enzymes.
Anemia:
. hemolytic nonspherocytic (spadkovy) type II
. due to hexokinase deficiency
. due to deficiency of pruvate kinase
. due to deficiency of triose phosphate isomerase
D55.3 Anemia due to disruption of nucleotide metabolism
D55.8 Other anemia due to enzyme disorders
D55.9 Anemia due to enzyme disorder, unspecified

D56 Thalassemia

D56.0 Alpha thalassemia.
Excludes: hydrops fetalis associated with hemolytic ailment (P56.-)
D56.1 Beta thalassemia. Anemia Kuli. Severe beta thalassemia. Sickle-clitin beta thalassemia.
Thalassemia:
. promizhna
. great
D56.2 Delta beta thalassemia
D56.3 Wearing signs of thalasemy
D56.4 Decreased persistence of fetal hemoglobin [NPFH]
D56.8 Other talases
D56.9 Thalassemia unspecified. Mediterranean anemia (with other hemoglobinopathy)
Thalassemia (small) (smishana) (with other hemoglobinopathy)

D57

Excludes: other hemoglobinopathy (D58.-)
sickle cell beta thalassemia (D56.1)

D57.0 Sickle cell anemia from crisis. Hb-SS ailment from crisis
D57.1 Sickle cell anemia without crisis.
Crescent-clitin(e):
. anemia)
. ailment)
. damaged)
D57.2 Subwines heterozygous sickle-clitin lesion
Ailment:
. Hb-SC
. Hb-SD
. Hb-SE
D57.3 Bearing signs of crescent-shaped cleavage. Carrying hemoglobin S. Heterozygous hemoglobin S
D57.8 Other sickle-clitin damage

D58 More progressive hemolytic anemia

D58.0 Falling spherocytosis. Aholurichna (simeina) Zhovtyanitsa.
Congenital (spherocytic) hemolytic zhovtyanitsya. Minkowski-Choffard syndrome
D58.1 Slumpy eliptocytosis. Ellitocytosis (congenital). Ovalocytosis (congenital) (spadkovy)
D58.2 Other hemoglobinopathy. Abnormal hemoglobin NOS. Congenital anemia due to Heinz's tilts.
Ailment:
. Hb-C
. Hb-D
. Hb-E
Hemolytic ailment is surrounded by non-stable hemoglobin. Hemoglobinopathy NOS.
Excludes: familial polycythemia (D75.0)
ailment Hb-M (D74.0)
decrease in persistence of fetal hemoglobin (D56.4)
polycythemia due to height (D75.1)
methemoglobinemia (D74.-)
D58.8 Other clarified cases of hemolytic anemia. stomatocytosis
D58.9 Spadkova hemolytic anemia, unspecified

D59 Prydbana hemolytic anemia

D59.0 Drug-induced autoimmune hemolytic anemia.
For consumption, identify the medicinal preparation with a supplementary code of external causes (class XX).
D59.1 Other autoimmune hemolytic anemias. Autoimmune hemolytic disease (cold type) (thermal type). Chronic ailment, which is called cold hemagglutinins.
"Kholodova aglyutinova":
. sickness
. hemoglobinuria
Hemolytic anemia:
. cold type (secondary) (symptomatic)
. thermal type (secondary) (symptomatic)
Excluded: Evans syndrome (D69.3)
hemolytic disease of the fetus and neonatal fetus (P55.-)
paroxysmal cold hemoglobinuria (D59.6)
D59.2 Drug non-autoimmune hemolytic anemia. Drug-induced enzyme deficiency anemia.
For the needs of identifying the medical zasib vikoristovuyut additional code of external reasons (class XX).
D59.3 Hemolytic-uremic syndrome
D59.4 Other non-autoimmune hemolytic anemias.
Hemolytic anemia:
. mechanical
. microangiopathic
. toxic
In times of need to identify the cause, add the additional code of the actual causes (class XX).
D59.5 Paroxysmal nocturnal hemoglobinuria [Markiafavi-Mikeli].
D59.6 Hemoglobinuria due to hemolysis caused by other external causes.
Hemoglobinuria:
. type of vanity
. marching
. paroxysmal cold
Excludes: haemoglobinuria NOS (R82.3)
D59.8 Other causes of hemolytic anemia
D59.9 Otriman's hemolytic anemia, unspecified. Idiopathic hemolytic chronic anemia

PLASTIC AND OTHER ANEMIA (D60-D64)

D60 Nabuta pure chervonoclitin aplasia (erythroblastopenia)

Inclusions: vermiform aplasia (pridbana) (mature) (with thymoma)

D60.0 Chronic nabuta pure chervonoklitina aplasia
D60.1 Passed the pridban pure chervonoklitinna aplasia
D60.8Інші pridbanі clean chervonoklitinnі aplazії
D60.9 Nabuta pure chervonoklitina aplasia unspecified

D61 Other aplastic anemia

Excluded: agranulocytosis (D70)

D61.0 Constitutional aplastic anemia.
Aplasia (pure) chervonoklitinna:
. congenital
. child
. pervinna
Blackfan-Diamond Syndrome. Simina hypoplastic anemia. Anemia Fanconi. Pancytopenia with vasodilatation
D61.1 Drug-induced aplastic anemia. If necessary, identify the medical zasib
vikoristovuyut additional code of ovnіshnіh causes (class XX).
D61.2 Aplastic anemia caused by other external agents.
In times of need to identify the cause, add the additional code of the actual causes (class XX).
D61.3 Idiopathic aplastic anemia
D61.8 Other clarified aplastic anemias
D61.9 Aplastic anemia, unspecified. Hypoplastic anemia NOS. Hypoplasia of the cystic brain. Panmieloftiz

D62 Gastrointestinal anemia

Excludes: congenital anemia due to fetal hemorrhage (P61.3)

D63 Anemia in chronic diseases, classified in other sections

D63.0 Anemia in neonates (C00-D48+)
D63.8 Anemia in other chronic ailments, classified under other headings

D64 More anemia

Excludes: refractory anemia:
. NOS (D46.4)
. too blast (D46.2)
. with transformation (D46.3)
. from sideroblasts (D46.1)
. without sideroblasts (D46.0)

D64.0 Spadkov's sideroblastic anemia. Hypochromic sideroblastic anemia was associated with the state
D64.1 Secondary sideroblastic anemia associated with other illnesses.
If necessary, identify the infection with a vicarious code.
D64.2 Secondary sideroblastic anemia caused by drugs and toxins.
In times of need to identify the cause, add the additional code of the actual causes (class XX).
D64.3 Other sideroblastic anemias.
Sideroblastic anemia:
. NOS
. pyridoxine-reactive, not classified in other sections
D64.4 Congenital dyserythropoietic anemia. Dyshemopoietic anemia (born).
Excluded: Blackfan-Diamond syndrome (D61.0)
ailment Di Guglielmo (C94.0)
D64.8 Other specified anemias. Childhood pseudoleukemia. Leukoerythroblastic anemia
D64.9 Anemia, unspecified

BLOOD MATERIALS, PURPLE AND INSHI

HEMORRHAGIC STATES (D65-D69)

D65 Disseminated intravascular larynx [defibrination syndrome]

Afibrinogenemia is present. coagulopathy
Diffuse or disseminated intravascular coagulation
Fibrinolytic bleeding of nabuta
Purpura:
. fibrinolytic
. bliskavichna
Exclusions: defibrination syndrome (what makes it worse):
. at the newly born (P60)

D66 Recessive factor VIII deficiency

Factor VIII deficiency (with functional impairment)
Hemophilia:
. NOS
. BUT
. classical
Excludes: factor VIII deficiency in vascular injury (D68.0)

D67 Recessionary factor IX deficiency

Christmas Pain
Deficit:
. factor IX (with functional impairment)
. thromboplastic component of plasma
Hemophilia B

D68 Other damage to the throat

Included: complicating:
. abortion, post-mortem or molar vagity (O00-O07, O08.1)
. vagity, sloping and post-sleep period (O45.0, O46.0, O67.0, O72.3)

D68.0 Ailment Willebrand. Angiohemophilia. Factor VIII deficiency in vascular injuries. Sudine hemophilia.
Excludes: fragility of congestive capillaries (D69.8)
factor VIII deficiency:
. NOS (D66)
. with functional impairment (D66)
D68.1 Falling deficiency of factor XI. Hemophilia C. Plasma thromboplastin anterior deficiency
D68.2 Recessionary deficiency of other factors of larynx. Congenital afibrinogenemia.
Deficit:
. AS-globulin
. proaccelerin
Factor deficiency:
. I [fibrinogen]
. II [prothrombin]
. V [labile]
. VII [stable]
. X [Stuart-Prower]
. XII [Hageman]
. XIII [fibrin-stabilizing]
Dysfibrinogenemia (congenital). Hypoproconvertinemia. Ovren's ailment
D68.3 Hemorrhagic damage, circulating in the blood with anticoagulants. Hyperheparinemia.
Promotion to change:
. antithrombin
. anti-VIIIa
. anti-IXa
. anti-Xa
. anti-XIa
If it is necessary to identify the vicorrhizae, the anticoagulant of the vicorist can be given an additional code of the underlying causes
(Class XX).
D68.4 Withdrawal deficiency of the laryngeal factor.
Deficiency of the laryngeal factor in the past:
. liver disease
. vitamin K deficiency
Exclusion: vitamin K deficiency in a neonate (P53)
D68.8 Other clarified damage to the larynx. The presence of an inhibitor of the systemic red dog
D68.9 Damaged throat, unspecified

D69 Purpura and other hemorrhagic conditions

Excludes: benign hypergammaglobulinemia purpura (D89.0)
cryoglobulinemia purpura (D89.1)
idiopathic (hemorrhagic) thrombocythemia (D47.3)
blissavich purpura (D65)
thrombotic thrombocytopenic purpura (M31.1)

D69.0 Allergic purpura.
Purpura:
. anaphylactoid
. Henocha(-Schönlein)
. non-thrombocytopenic:
. hemorrhagic
. idiopathic
. sudinna
allergic vasculitis
D69.1 Yakіsnі nedolіki trombotsiіv. Bernard-Soullet [giant platelets] syndrome.
Glanzman's ailment. Syndrome of orphan thrombocytes. Thrombasthenia (hemorrhagic) (spadkova). Thrombocytopathy.
Excluded: Willebrand's disease (D68.0)
D69.2 Other non-thrombocytopenic purpura.
Purpura:
. NOS
. senile
. simple
D69.3 Idiopathic thrombocytopenic purpura. Evans syndrome
D69.4 Other primary thrombocytopenia.
Inclusion: thrombocytopenia with rupture of the promeneutic cyst (Q87.2)
past neonatal thrombocytopenia (P61.0)
Wiskot-Aldrich syndrome (D82.0)
D69.5 Secondary thrombocytopenia. In times of need to identify the cause, add the additional code of the actual causes (class XX).
D69.6 Thrombocytopenia, unspecified
D69.8 Other clarified hemorrhagic stages. Brittleness of capillaries (spadkov). Sudin pseudohemophilia
D69.9 Hemorrhagic camp unspecified

ІНШІ BLOOD DISEASES AND BLOOD ORGANITS (D70-D77)

D70 Agranulocytosis

Agranulocytic angina. Childhood genetic agranulocytosis. Pain Kostmann
Neutropenia:
. NOS
. congenital
. cyclical
. medication
. periodic
. selezinkova (pervinna)
. toxic
Neutropenic splenomegaly
If it is necessary to identify the medical condition, which caused neutropenia, the additional code of external causes (class XX) should be given.
Excluded: past neonatal neutropenia (P61.5)

D71 Functional impairment of polymorphonuclear neutrophils

Defect of the receptor complex of the cellular membrane. Chronic (childish) granulomatosis. Natural dysphagocytosis
Progressive septic granulomatosis

D72 Other damage to white blood cells

Excluded: basophilia (D75.8)
immune disorders (D80-D89)
neutropenia (D70)
preleukemia (syndrome) (D46.9)

D72.0 Genetic anomalies of leukocytes.
Anomaly (granulation) (granulocyte) or syndrome:
. Aldera
. Travnya-Kheґґlina
. Pelgera Huet
Spadkov:
. leukocytic
. hypersegmentation
. hyposegmentation
. leukomelanopathy
Excludes: Chediak-Higasi (-Steinbrink) syndrome (E70.3)
D72.1 Eosinophilia.
Eosinophilia:
. allergic
. spadkov
D72.8 Other clarified damage to white blood cells.
Leukemoid reaction:
. lymphocytic
. monocytic
. myelocytic
Leukocytosis. Lymphocytosis (symptomatic). Lymphospenia. Monocytosis (symptomatic). plasmacytosis
D72.9 Destruction of two blood cells, unspecified

D73 Spleen ailments

D73.0 Hyposplenism. Asplіnnya postoperative. Atrophy of the spleen.
Excludes: asplenia (congenital) (Q89.0)
D73.1 Hypersplenism
Excludes: splenomegaly:
. NOS (R16.1)
.born (Q89.0)
D73.2 Chronic congestion splenomegaly
D73.3 Abscess of the spleen
D73.4 Cyst of the spleen
D73.5 Spleen infarction. The rupture of the spleen is non-traumatic. Torsion of the spleen.
Inclusion: traumatic rupture of the spleen (S36.0)
D73.8 Other ailments of the spleen. Fibrosis of the spleen NOS. Respawn. Split NOS
D73.9 Spleen ailment, unspecified

D74 Methemoglobinemia

D74.0 Born methemoglobinemia. Congenital deficiency of NADH-methemoglobin reductase.
Hemoglobinosis M [Hb-M ailment]. Spadkov's methemoglobinemia
D74.8 Other methemoglobinemia. Nabuta methemoglobinemia (from sulfhemoglobinemia).
Toxic methemoglobinemia. In times of need to identify the cause, add the additional code of the actual causes (class XX).
D74.9 Methemoglobinemia, unspecified

D75 Other diseases of the blood and hematopoietic organs

Excludes: enlargement of lymph nodes (R59.-)
hypergammaglobulinemia NOS (D89.2)
lymphadenitis:
. NOS (I88.9)
. hostrium (L04.-)
. chronic (I88.1)
. Brizhovy (hostry) (chronic) (I88.0)

D75.0 Family erythrocytosis.
Polycythemia:
. good-natured
. family
Excluded: recessive ovalocytosis (D58.1)
D75.1 Secondary polycythemia.
Polycythemia:
. pridbana
. pov'yazana z:
. erythropoietins
. reduced plasma pressure
. curls
. stress
. emotional
. hypoxemic
. nephrogenic
. visible
Excludes: polycythemia:
. newly born (P61.1)
. true (D45)
D75.2 Essential thrombocytosis.
Excludes: essential (hemorrhagic) thrombocythemia (D47.3)
D75.8 Other clarified diseases of the blood and hematopoietic organs. Basophilia
D75.9 Blood ailment and hematopoietic organs unspecified

D76 Severe ailments that flow from irradiated lymphoreticular tissue and the reticulo-stiocytic system

Excludes: Letterer-Cive disease (C96.0)
malignant histiocytosis (C96.1)
reticuloendotheliosis or reticulosis:
. histiocytic medullary (C96.1)
. leukemic (C91.4)
. lipomelanotic (I89.8)
. malicious (C85.7)
. nonlipid (C96.0)

D76.0 Histiocytosis from Langerhans cells, not classified in other rubrics. Eosinophilic granuloma.
Ailment Hand-Schuller-Chrisgen. Histiocytosis X (chronic)
D76.1 Hemophagocytic lymphohistiocytosis. Familial hemophagocytic reticulosis.
Histiocytosis from mononuclear phagocytes, Langerhans cletin species, NOS
D76.2 Hemophagocytic syndrome, involvement from infection.
If it is necessary to identify the infectious disease, the sickness of the vicorist must be given an additional code.
D76.3 Other histiocytosis syndromes. Reticulohistiocytoma (giantoclitin).
Sinus histiocytosis due to massive lymphadenopathy. Xanthogranuloma

D77 Other disorders of the blood and hematopoietic organs in diseases classified under other headings.

Fibrosis of the spleen in schistosomiasis [Bulharzia] (B65.-)

DAMAGES THAT INCLUDE THE IMMUNE MECHANISM (D80-D89)

Inclusions: defects in the complement system, immunodeficiency disorders,
human immunodeficiency virus [VIL] sarcoidosis
Excludes: autoimmune ailments (systemic) NOS (M35.9)
functional impairment of polymorphonuclear neutrophils (D71)
ailment caused by human immunodeficiency virus [HIV] (B20-B24)

D80 Immunodeficiency with significant antibody deficiency

D80.0 Spadkova hypogammaglobulinemia.
Autosomal recessive agammaglobulinemia (Swiss type).
X-linked agammaglobulinemia [Bruton's] (growth hormone deficiency)
D80.1 Non-similar hypogammaglobulinemia. Agamaglobulinemia with the presence of B-lymphocytes, which are carried by immunoglobulins. Global agammaglobulinemia. Hypogammaglobulinemia NOS
D80.2 Viboric deficiency of immunoglobulin A
D80.3 Selective deficiency of subclasses of immunoglobulin G
D80.4 Viboric deficiency of immunoglobulin M
D80.5 Immunodeficiency due to promotion of immunoglobulin M
D80.6 Deficiency of antibodies due to close to normal levels of immunoglobulins or hyperimmunoglobulinemia.
Antibody deficiency with hyperimmunoglobulinemia
D80.7 Past hypogammaglobulinemia in children
D80.8 Other immunodeficiencies with a major antibody defect. Kappa lung lanceg deficiency
D80.9 Immunodeficiency with major antibody defect, unspecified

D81 Combined immunodeficiency

Excludes: autosomal recessive agammaglobulinemia (Swiss type) (D80.0)

D81.0 Severe combination of immunodeficiency with reticular dysgenesis
D81.1 Severe combination immunodeficiency with low T- and B-cell counts
D81.2 Severe combination immunodeficiency with low or normal levels of B-cellin
D81.3 Adenosine deaminase deficiency
D81.4 Nezelof syndrome
D81.5 Purine nucleoside phosphorase deficiency
D81.6 Deficiency of class I molecules of the histosum head complex. Naked lymphocyte syndrome
D81.7 Deficiency of class II molecules of the histosum head complex
D81.8 Other combinations of immunodeficiency. Deficiency of biotin-depleted carboxylase
D81.9 Combination immunodeficiency not specified. Severe combination immunodeficiency disorder NOS

D82 Immunodeficiencies associated with minor defects

Excludes: atactic telangiectasia [Louis Bar] (G11.3)

D82.0 Wiskott-Aldrich syndrome. Immunodeficiency with thrombocytopenia and eczema
D82.1 Di George syndrome. Gorilla diverticulum syndrome.
Vilochkov's lash:
. alimphoplasia
. aplasia or hypoplasia with immune deficiency
D82.2 Immunodeficiency with dwarfism due to short term fluke
D82.3 Immunodeficiency due to a recessive defect caused by the Epstein-Barr virus.
X-linked lymphoproliferative ailment
D82.4 Hypermunoglobulin E syndrome
D82.8 Immunodeficiency, involvement with other specified significant defects
D 82.9 Immunodeficiency, involvement with a significant defect, unspecified

D83 Primary variable immunodeficiency

D83.0 Severe variable immunodeficiency with predominant respiratory impairment and functional activity of B-clitin
D83.1 Severe variable immunodeficiency due to overwhelmed disorders of immunoregulatory T-cells
D83.2 Global Variable Immunodeficiency with Autoantibodies to B- or T-Clitin
D83.8 Other globally variable immunodeficiencies
D83.9 Global variable immunodeficiency, unspecified

D84 Other immunodeficiencies

D84.0 Defect of functional antigen-1 lymphocytes
D84.1 Defect in the complement system. Deficiency of C1 esterase inhibitor
D84.8 Other clarified immunodeficiency disorders
D84.9 Immunodeficiency, unspecified

D86 Sarcoidosis

D86.0 Sarcoidosis Legion
D86.1 Sarcoidosis of the lymph nodes
D86.2 Sarcoidosis of the leg with sarcoidosis of the lymph nodes
D86.3 Sarcoidosis shkiri
D86.8 Sarcoidosis of other clarifications and combined localizations. Iridocyclitis in sarcoidosis (H22.1).
Multiple cranial nerve palsies in sarcoidosis (G53.2)
Sarcoidna(s):
. arthropathy (M14.8)
. myocarditis (I41.8)
. myositis(M63.3)
Uveoparotitna lihomanka [Gerfordt's ailment]
D86.9 Sarcoidosis unspecified

D89 Other disorders due to radiation of the immune mechanism, not classified in other sections

Excludes: hyperglobulinemia NOS (R77.1)
monoclonal gammopathy (D47.2)
graft failure and rejection (T86.-)

D89.0 Polyclonal hypergammaglobulinemia. Hypergammaglobulinemia purpura. Polyclonal gammopathy NOS
D89.1 Cryoglobulinemia.
Cryoglobulinemia:
. essential
. idiopathic
. zmishana
. pervinna
. secondary
Cryoglobulinemia(s):
. purpura
. vasculitis
D89.2 Hypergammaglobulinemia, unspecified
D89.8 Other clarified damage due to radiation of the immune mechanism, not classified in other headings
D89.9 Destruction, as if catching the immune mechanism, not specified. Immune ailment NOS

Hemolytic anemia tends to develop in one's own group of ailments, which may have a spontaneous nature. These illnesses are characterized by an accelerated collapse of red bodies, the result is a significant increase in the amount of indirect bilirubin. Over ten hundred years of anemia can lie on its own to a hemolytic form. For this disorder, absolutely all people are sick, it is necessary to know the main symptoms, the causes and methods of healing this disorder.

The etiology of hemolytic anemia can be seen in congestive and swollen diseases. And the reason for the development of the ailment of varto shukati is not less in the blood-bearing system, but in other systems of the body. Hemolytic anemia develops due to such causes:

1. Penetration into the blood of various toxic speeches and blast chemicals, they can also be bitten by blast creatures.
2. With mechanical collapse of erythrocytes.
3. Genetic factor, which has contributed to the anomaly in budovі erythrocytes.
4. As a result of infection on an infectious disease.
5. Avitaminosis.
6. Great injuries, strong ops and operational intervention.

Unfortunately, after identifying the causes of hemolytic anemia, it is not possible to prevent the progression of the disease in patients. Deyakі provocative appearances do not zavzhda vdaetsya usunut, scho becomes a worsening factor in symptoms.

Symptoms of hemolytic anemia

Signs of hemolytic anemia occur in two main syndromes - anemic and hemolytic. The clinical picture of the anemic syndrome is more pronounced in manifestations of the onset of symptoms: pale eyesight and mucous membranes, increased stupor, partial confusion, the appearance of a buttocks in the case of insignificant pressures, an accelerated heartbeat. The picture of symptoms in case of hemolytic factor may still manifest: yellowish-bluish appearance of the scrotum, dark brown urine, enlarged spleen size, more discomfort in the left subcostal area.

All advances in the progression of illness are manifested by such signs: there is an increase in weakness in the whole body and headache, pain can be seen in other parts of the body, fever and vomit appear. Also, dark-chervony urine is fixed. Severe hemolytic anemia is manifested by an increase in body temperature.

The pathogenesis of hemolytic anemia

Mechanism for the development of illness without intermediary treatment with an accelerated collapse of erythrocytes, which comes through the damage to the integrity of the exoskeleton. Hemolytic effect of influx of toxic speech and non-intermediate infusion on all main storage membranes. The hemolytic effect develops due to the development of pathological oxidation, which sprays the accumulated peroxide from the large number.

This pathological mechanism should be brought to the point where there is a functional and structural change in the hemoglobin storage, various damage in the membrane storage of erythrocytes. Sometimes it is possible to predict a secondary hemolytic effect, which is called by some chemical speeches. Trivaliy negative infusion of these speeches is a whole building disease and a chronic overflow of hemolytic anemia. The pathogenesis of this disease can be changed and folded, which will require detailed and deep investigation.

It is precisely possible to determine that damage to the structural membrane of erythrocytes, as well as functional damage, lead to the fact that the processes of life of erythrocytes change, which also affects the trivality of their foundation. Basically, hemolytic anemia is caused through a non-professional factor, zocrema in women.

Hemolytic anemia in children

Pathological disease of the blood in children of mature age is characterized by a characteristic overrun of anemia and cyclical features - in children, a change in the period of acute and remission occurs. In children, hemolytic anemia is of great concern - for the nervous system, and more precisely for the brain. In young children, most often there is a zhovtyanichny zabarvlennya skirnih crooked.

New people and children of preschool age suffer from all kinds of anemia due to various causes. The most common reason for this is the recessionary factor, which manifests itself especially in the new people. Another common problem is blood transfusion, if it is not necessary. In children, anemia is caused by the fact that the mother took the same drug preparations for the hour of pregnancy.

Other provocateurs include toxic poisoning with lead, a bruising bite of a coma or a snake, autoimmune damage that leads to a collapse of the erythrocyte membrane, a disease of an infectious nature, severe opiates, traumatic situations, cold weather. In addition, as the disease develops, new signs of pathology and other serious symptoms appear. ICD code 10 for hemolytic anemia can have two meanings: D58 - less relapsing hemolytic anemia, and also code D59 - advanced hemolytic anemia.

Classification of hemolytic anemias

Congenital hemolytic anemia can be subdivided into the following forms: erythrocytopathy and hemoglobinopathy. The first form is similar to congenital spherocytic and non-spherocytic anemia, and to induce a severe form, as a result of medicamentous officials with viruses. Here lie chronic hemolytic anemia. Other forms include thalassemia and sickle cell anemia.

Nabuty different types of anemia are fixed in such forms: gostra and chronic. The host form manifests itself as a result of a disease of a newborn, an infectious disease through a blood transfusion. The chronic form is prevented through autoimmune disease or other chronic diseases.

Nabuta hemolytic anemia

Two groups of hemolysis are divided into two groups of hemolysis: congenital anemia and nabuta. In order to better understand the mechanisms of elimination and the development of the developed form of hemolytic anemia, it is necessary to better understand the causes, the main symptoms and the best tactics for the treatment of this variety. This form of anemia in a child is accompanied by a greater manifestation of illness.

For this form of anemia, a characteristic rise in the presence of an influx of external or internal provocateurs, which is absolutely not related to erythrocytes. It is problematic to make an accurate diagnosis in an early age in a child. This is due to the fact that the blood of the new people is not more stable in power and physical features. So varto signify, deyakі fahivtsі do not know that there is a form of anemia.

Immune hemolytic anemia

Lіkarska hemolytic anemia can become twenty years old in case of successful types of hemolytic anemias. In different cases of illness, it is noted that hemolysis is manifested only for a few hours of the first drug, which is often used after taking the drug. Clinical signs should also be manifested: blistering disease, yellowness, improvement in internal organs, sickness, zadishka.

The hematologist is engaged in the diagnosis of the form of hemolytic anemia with the correction of blood tests, causes, symptoms of symptoms. At the first glance, the anamnesis is revealed. We will then carry out an assessment of the contamination of the skin curves, visible mucous membranes. The process of diagnostics polіgaє at the last equal bilirubin.

The Coombs test is used to identify clinical and hematological signs of hemolysis, as well as to detect autoantibodies on the surface of erythrocytes. In some cases, blood tests for hemolytic anemia should indicate the presence of microspherocytes, and laboratory diagnostics of hemolytic anemia is sometimes necessary. Also, the rіven SHOE is significantly moving, platelets, most often, change at the borders of the norm. Analyzes in hemolytic anemia show an increase in bilirubin in the blood.

Treatment of hemolytic anemia

The symptoms of hemolytic anemia are determined by the degree of severity of the illness. Mustache forms of difference for the tactics of glee, shards toil their own particularity. However, one thing is left with the inevitable for whatever form of illness - the first thing you need to do is to take up the negative injection of factors that hemolyze. All patients are prescribed blood plasma, necessary vitamins, in other cases - hormonal therapy, taking antibiotics. Splenectomy is considered the only effective way to treat hemolysis in microspherocytosis.

The autoimmune form of this disease is treated with the use of glucocorticoid hormonal preparations, which can lead to rapid or complete hemolysis. In okremy cases of obov'yazykovy part of drug therapy, the recognition of immunosuppressants and antimalarial diseases. Toxic hemolytic anemia is treated with intensive therapy: detoxification, diuresis, antidote. What is the cost of nirkov's lack fixes an unacceptable life prognosis.

RCHD (Republican Center for the Development of Health Protection of the Ministry of Health of the Republic of Kazakhstan)
Version: Clinical protocols of the Ministry of Health of the Republic of Kazakhstan - 2016

Other autoimmune hemolytic anemia (D59.1), Drug-induced autoimmune hemolytic anemia (D59.0)

Orphan's illness

General information

Short description

toppled
Joint commission for the quality of medical services
Ministry of Health and Social Development of the Republic of Kazakhstan
date "15" spring 2016
Protocol #11

Autoimmune hemolytic anemia (AIHA)- a heterogeneous group of autoaggressive disease syndromes, leading to the collapse of erythrocytes, which is caused by uncontrolled production of antibodies against erythrocytes.

ICD-10 and ICD-9 code spiving:

MKL-10		MKL-9
The code	name	The code	name
D59.0	Drug-induced autoimmune hemolytic anemia	283.0	Autoimmune hemolytic anemia
D59.1	Other autoimmune hemolytic anemias Autoimmune hemolytic disease (cold type) (thermal type) Chronic ailment, which is called cold hemagglutinins "Cold agglutinin": ailment. Hemoglobinuria Hemolytic anemia: . cold type (secondary) (symptomatic). heat type (secondary) (symptomatic) Excludes: Evans syndrome (D69.3) hemolytic fetal and neonatal disease (P55.-) paroxysmal cold hemoglobinuria (D59.6)

Date of release / revision of the protocol: 2016 rec.

Koristuvachi protocol: doctors of Swedish medical assistance, doctors of general practice, therapists, hematologists.

Evidence scale:

A	A high-level meta-analysis, a systematic review of the Republic of Kazakhstan or a great Republic of Kazakhstan with an even lower (++) systematic pardon, the results of which can be extended to a specific population.
B	High (++) systematic review of cohort or lingering decline-control or High (++) cohort or lingering decline-control with an even low risk of systematic pardoning or RKI with a low (+) risk of systematic pardoning, the results of which can be broadened in the population .
C	Cohort or follow-up drop-control or controlled follow-up without randomization with a low risk of systematic pardon (+). The results of any of them can be expanded on a specific population or RKD with an even low or low risk of a systematic pardon (++ or +), the results of which cannot be broadly expanded on a specific population.
D	Description of a series of behaviors that are not controlled by the opinion of experts.

Classification

Classification:
AIHA can be divided into idiopathic (primary) and symptomatic (secondary). More lower in 50% of patients, the development of AIHA may be secondary (Table 1).
In 10% of patients with AIHA, the cause of hemolysis is various drug preparations. The list of preparations that can lead to the development of autoimmune hemolysis or lead to the detection of anti-erythrocyte antibodies - div. addition 1.

The serological power of the autoantibodies formed the basis for the subdivision of AIHA on chotiri form:
· Incomplete thermal agglutinins (80% of all diseases);
· With more cold agglutinins (12-15% of all fluctuations);
3 thermal hemolysins;
· with two-phase cold hemolysins of Donat-Landsteiner (rarely rare and, as a rule, a secondary form in syphilis and viral infections).

Table 1 - Frequency of this type of antibody in secondary AIHA

Ill chi camp *	AIHA frequency, %	AIHA with thermal autoantibodies	AIHA with cold autoantibodies
HLL	2.3-4.3	87%	7%
NHL (Crimea HLL)	2,6	More often	m
IgM gammopathy	1,1	no	mustache
Hodgkin's lymphoma	0,19-1,7	Mayzhe all	rarely
Solid fluff	Duzhe rarely	2/3	1/3
Dermoid cyst of the ovaries	Duzhe rarely	mustache	no
GVH	6,1	Mayzhe all	rarely
Nonspecific virazkovy colitis	1,7	mustache	no
	5,5	mustache	no
	50	mustache	no
After allogeneic TCM	44	So	So
After organ transplantation	5.6	So	no
Drug-induced in CLL	2.9-10.5 very rare	Mayzhe all	rarely
Interferon	Frequency 11.5/100,000 patient-births	mustache	no

Diagnostics (ambulatory)

DIAGNOSTICS AT THE OUTPATIENT RIVNI (UD - V)

Diagnostic criteria:

Skargi and history:
The main syndromes in hemolytic anemiasє:
· Normocytic anemia with rapidly increasing weakness and nasty adaptation to lead to a deathly decrease in hemoglobin.

Depending on the level of hemoglobin, 3 degrees of severity of anemia are seen:
· I (easy step) - Hb more than 90 g/l;
II (middle stage) - vіd 90 to 70 g/l;
III (important step) - less than 70 g / l.

Clinically, the severity of the patient's condition does not change depending on the level of hemoglobin: anemia, which has developed acutely, is accompanied by significantly more severe symptoms, lower chronic, with an hour for adaptation of organs and tissues. Older patients suffer from anemia older, younger, little compensatory capacity of the cardiovascular system, as a rule, is reduced.

In hemolytic crisis, signs of severe anemia are manifested on aphids of the acute cob:
fever fever;
pain in the abdomen;
Head bіl;
· Blue water;
· oliguria and anuria with a further development of shock.

Hemolysis syndrome, which can be manifested by scars on:
Іkterichnost shkіri and visible mucous membranes (zhovtukha);
· Darkening section.
In case of intravascular hemolysis, the color of the section can be from erysipelas to black. The color is deposited according to the concentration of hemoglobin, the degree of dissociation of the heme. The color of the section in case of hemoglobinuria is necessary to be examined in the presence of hematuria, if the number of erythrocytes is visible in the microscopic examination. The color of the sechi can be chervonim through the use of medicinal preparations (antipirin), zhі (buryakіv) or with porphyria, myoglobinuria, as it develops in singing camps (massive traumatic injury, electrical shock, arterial thrombosis and others).
· Appearing sensitiveness when pressed, feeling the severity or pain in the left subcostal area, connected with the enlarged spleen. Most of the steps in the improvement of the spleen may have an insignificant, morbid character.

More low in 50% of patients, the development of AIHA may be of a secondary nature, in connection with which the clinical picture may be dominated by symptoms of the main disease (Table 1).

Physically obstezhennya:
The results of physical obstezhennja are determined by the rate and stage of hemolysis, manifestation or comorbidity, illness, which caused the development of AIHA. At the stage of compensation, the state is overdone, there may be slight subicterism of the skin curves, visible mucous membranes, splenomegaly is small, signs of the main illness, for example, VKV, lymphoproliferative illness, etc. In this situation, the presence of lung AIHA may not be diagnosed.

In hemolytic crisis:
· Stan average gravity or heavy;
· blіdіst shkіri and mucus;
Expansion between the heart, deafness of tones, tachycardia, systolic murmur at the top;
· Zadishka;
Weakness;
· Zamorochennya;
· bilirubin intoxication: ictericity of the skin and mucous membranes, nudota, vomit, pain in the abdomen, confusion, head pain, lihomania, in some cases, the disorder of svіdomostі, sudomi;
· with intracellular hemolysis: hepatosplenomegaly;
· in case of mixed and intravascular hemolysis: change of cross section for hemoglobinuria.

Laboratory studies:
· Global blood analysis, including platelets and reticulocytes: anemia of varying degrees of severity is normochromic; reticulocytosis, leukocytosis with damage to the leukocyte formula to the left during the crisis; in a smear of peripheral blood, as a rule, microspherocytes;
· Biochemical analysis of blood:
bilirubin with fractions (hyperbilirubinemia, passing indirectly, non-conjugated fraction),
· LDH (increased activity of LDH in syrovattsi by 2-8 times in fallow due to the intensity of hemolysis),
haptoglobin - indicative of hemolysis;
burning protein, albumin, creatinine, sechovin, ALT, AST, GGTP, C-reactive protein, phosphatase pool - assessment of the liver, nirok
Glucose - blame for diabetes;
Direct Coombs' test in most cases - positive, but with massive hemolysis, as well as with cold and hemolysin forms of AIHA, IgA or IgM autoantibody, may be negative.


· hemosiderin in the section - the culprit of intravascular hemolysis;
· hot analysis section (obov'yazkova visual assessment of the color of the section);
· Significance of midi in the dobovіy section, ceruloplasmin in the blood serum - exclusion of Wilson-Konovalov's ailment;
Puncture of the cystic brain (hyperplasia and morphology of the erythroid pariet, number and morphology of lymphocytes, complexes of metastatic cells);
trepanobiopsia (if necessary) - exclusion of secondary AIHA;
Immunophenotyping of lymphocytes (with lymphocytosis of peripheral blood and distant spleen) - exclusion of secondary AIHA;
· vitamin B12, folate - the cause of megaloblastic anemia;
· Indications of the exchange of saliva (including transferrin, feritin of sirovatka and erythrocytes) - blames for salivary deficiency;
· Broken coagulogram + Vovchakovy anticoagulant - assessment of hemostasis, APS vignettes;
rheumatological tests (antibodies to native DNA, rheumatoid factor, antinuclear factor, antibodies to cardiolipin antigen) - exclusion of secondary AIHA;

· If necessary - hormones of the thyroid gland, prostatic specific antigen, tumor markers-exclusion of secondary AIHA;
· Designation of blood group for the AB0 system, Rh factor;
· Blood test for VIL - for the need for transfusion;
· Blood test for syphilis - standard obstezhennia for any equal;
· Determination of HBsAg in blood serum by ELISA method - screening for hepatitis B;
· Identification of total antibodies to hepatitis C virus (HCV) in blood syrometry by ELISA method - screening for hepatitis C.

Instrumental follow-up:
X-ray legenography (if necessary, CT);
FGDS;

Ultrasound of organs empty stomach and intracerebral lymph nodes, small pelvis, anterior fold, thyroid fold.

Diagnostic algorithm (Scheme 1):

Diagnostics

DIAGNOSIS AND LIKUVANNYA AT THE STAGES OF SVIDKY INVISIBLE AID

Diagnostic login:
· Zbіr skarg, anamnesis;
· Physically obstezhennya.

Medication treatment: no.

Diagnostics (hospital)

DIAGNOSIS ON STATIONARY RIVNI

Diagnostic criteria: div. outpatient rіven.

Diagnostic algorithm: div. outpatient rіven.

A list of the main diagnostic approaches:
· global blood analysis (pidrachunok leukoformula, thrombocytes and reticulocytes in a smear);
· Biochemical analysis of blood (hard bilirubin, direct bilirubin, LDH);
Direct Coombs test.

A list of additional diagnostic approaches:
· Appointment equal to haptoglobin;
· Blood group and Rh factor;
Biochemical analysis of blood (salt protein, albumin, seperate bilirubin, direct bilirubin, creatinine, sechovin, ALaT, ASAT, glucose, LDH, GGTP, C-reactive protein, phosphatase puddle);
exchange of saliva (significance of the rіvnya of the sirovatkovy rіlіz, the rіvnіnja rіvnа of the sirovatkovy rіzіz, the rіvnії rіvnja sіvіychoї zdatnostі syrovatki і rіvіn feritin);
· Designated concentration of folic acid and vitamin B12;
Immunophenotyping of lymphocytes (with lymphocytosis, suspected lymphoproliferative disease, ineffectiveness of therapy with corticosteroids);
Electrophoresis of whites of the shrimp and sections with immunofixation (with lymphocytosis, suspected lymphoproliferative disease, ineffectiveness of therapy with corticosteroids);
Myelogram;
ELISA for markers of viral hepatitis;
· IFA for VIL markers;
· IFA on markers of viruses of herpes-group;
Coagulogram, Vovchakovy anticoagulant;
Rehberg-Tarєєva test (designation of glomerular filtration rate);
The titer of cold agglutinins;
Indirect Coombs' test (obov'yazkova with intensive hemolysis and forward transfusions of erythrocytes);
· Designation of hemosiderin, midi and hemoglobin in section;
trepanobiopsy of the cystic brain with histological findings;
vitamin B12, folate;
· Indications of the exchange of saliva (including transferrin, feritin of sirovatka and erythrocytes);
Coagulogram + Vovchakovy anticoagulant;
rheumatological tests (antibodies to native DNA, rheumatoid
· Factor, antinuclear factor, antibodies to cardiolipin antigen);
· immunoglobulins of sirovatka (G, A, M) + cryoglobulins;
· Hormones of the thyroid gland, prostatic specific antigen, tumor markers;
· hot analysis of the section;
X-ray of organs of the chest;
Esophagogastroduodenoscopy;
Irigoscopy / sigmoidoscopy / colonoscopy;
· Ultrasound of the organs of the empty cranial and intracerebral lymph nodes, small pelvis, anterior fold, thyroid duct;
· Ultrasound of arteries and veins;
· ECG;
Echocardiography;
· Additional monitoring of AT;
· Further monitoring of the ECG.

Differential diagnosis

Differential diagnosis and obstruction of additional findings:

Diagnosis	Priming for differential diagnosis	obstezhennya	Criteria for confirming the diagnosis
AIGA with incomprehensible thermal agglutinins (primary)	Presence of anemia, hemolysis	Direct Coombs test, puncture of the cystic brain (hyperplasia and morphology of the erythroid pariet, number and morphology of lymphocytes, complexes of metastatic cells); immunophenotyping of lymphocytes (with lymphocytosis of peripheral blood and distant spleen); rheumatological tests (antibodies to native DNA, rheumatoid factor, antinuclear factor, antibodies to cardiolipin antigen); immunoglobulins of sirowort (G, A, M) + cryoglobulins; thyroid hormones, prostatic specific antigen, tumor markers); . Ultrasound examination of the organs of the empty cranial and intracerebral lymph nodes, the small pelvis, the anterior fold, the thyroid tract; . radiography legenia (for the need for CT); colonoscopy	positive direct Coombs test, evidence for the second nature of anemia
AIHA with more cold agglutinins		titer of cold agglutinins; wild analysis of the section (obov'yazkovaya є visual assessment of the color of the section); prescription of hemosiderin immunoglobulin (G, A, M) + cryoglobulin;	In clinical picture, intolerance to cold (blue, and then bubbling of the fingers, nig, vuh, tip of the nose, sharp pain in the tips), seasonality of illness. In case of obstruction-impossibility of the blood group and erythrocytes, the appearance of an M-gradient, a high titer of cold antibodies at t 4 0
Falling hemolytic anemia	Presentation of anemia, hemolysis syndrome	Direct Coombs' test, ultrasound of mucilage, spleen, morphology of erythrocytes, if necessary - identification of enzyme activity in erythrocytes, electropheresis of hemoglobin	Anamnesis of childhood, severe depression, when looking at signs of embryogenesis, negative direct Coombs test
B12 deficiency anemia	Presentation of anemia, hemolysis syndrome	Follow-up to vitamin B12	Funicular myelosis, decreased vitamin B12 Negative direct Coombs test
Wilson's disease	Presentation of anemia, hemolysis syndrome in the onset of illness	Direct Coombs test, investigation of midi in the section, ceruloplasmin in the blood, consultation of a neuropathologist, oculist	Signs of damage to the nervous system, liver, presence of the Kaiser-Fleischer ring, decrease in the level of ceruloplasmin in blood plasma, decrease in midi in blood plasma, increased excretion of midi in the cross section
PNG	Presentation of anemia, hemolysis syndrome	Immunophenotyping of peripheral blood for detection of PNH erythrocyte types I, II and III by flow cytometry	test tsukrozna that Hema is positive; immunophenotyping-expression of GPI-surfaces of proteins; the patient's sucrose does not cause hemolysis of the donor's erythrocytes

Jubilation beyond the cordon

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Jubilation

Preparations (dіyuchі speechovini), scho zastosovuyutsya with likuvanni

Azathioprine (Azathioprine)

Alemtuzumab (Alemtuzumab)

Olendronic acid (Alendronic acid)

Alfacalcidol (Alfakaltsidol)

Amikacin (Amikacin)

Amlodipine (Amlodipine)

Amoxicillin (Amoxicillin)

Atenolol (Atenolol)

Acyclovir (Acyclovir)

Valacyclovir (Valacyclovir)

Valganciclovir (Valganciclovir)

Water for injections (Water for Injection)

Ganciclovir (Ganciclovir)

Dextrose (Dextrose)

Dopamine (Dopamine)

Drotaverine (Drotaverinum)

Zoledronic acid (Zoledronic Acid)

Imipenem (Imipenem)

Potassium chloride (Potassium chloride)

Calcium carbonate (Calcium carbonate)

Captopril (Captopril)

Ketoprofen (Ketoprofen)

Clavulanic acid

Levofloxacin (Levofloxacin)

Lisinopril (Lisinopril)

Mannitol (Mannitol)

Meropenem (Meropenem)

Methylprednisolone (Methylprednisolone)

Mycophenolic acid (Mycophenolate mofetil) (Mycophenolic acid (Mycophenolate mofetil))

Nadroparin calcium (Nadroparin calcium)

Sodium chloride (Sodium chloride)

Nebivolol (Nebivolol)

Omeprazole (Omeprazole)

Paracetamol (Paracetamol)

Pneumococcal vaccine

Prednisolone (Prednisolone)

Rabeprazole (Rabeprazole)

Risedronic acid (Risedronic acid)

Rituximab (Rituximab)

Torasemid (Torasemide)

Famciclovir (Famciclovir)

Fluconazole (Fluconazole)

Folic acid (Folic acid)

Chloropiramine (Chloropyramine)

Cyclosporine (Cyclosporine)

Cyclophosphamide (Cyclophosphamide)

Ciprofloxacin (Ciprofloxacin)

Enoxaparin sodium (Enoxaparin sodium)

Erythrocyte dependence leukofiltrated

Groups of drugs that are beneficial for ATC, which should be included in the treatment

Likuvannya (outpatient clinic)

LIKUVANNYA AT THE OUTPATIENT RIVNI (UD - V)

Tactics of jubilation: only for daily indications before hospitalization: at the outpatient stage, treatment is often continued, at the hospital, monitoring of clinical and laboratory indications and further correction of therapy.

Non-drug treatment:
ModeII. With triple therapy with corticosteroids, regular physical right, the risk factors for depression are equal, falling (C), and smoking. With AIHA with cold antibodies, there is no overcooling.
Diet: for the prevention of glucocorticoid osteoporosis, it is adequate to reduce calcium and vitamin D, to reduce alcohol (D).

Medication treatment:

Prednisolone;

· Rituximab concentrate for preparation for infusion 100 mg;
· Cyclosporine;
Amlodipine;
lisinopril;
atenolol;
Torasemide;
folic acid;
alendronate;
risedronate;
· Zolendronate;
Alfacalcidol;
calcium carbonate;
· Paracetamol;
chlorpyramine;
Omeprazole;
Enoxaparin;
· Nadroparin;
Amoxicillin/clavulanic acid;
Levofloxacin;
· Rosen sodium chloride.

AIGA therapy in this hour is not based solely on the basis of retrospective and number of prospective studies for the number of randomized studies and not a high level of evidence. Also, there are daily formal consensus about the appointment of a new or private remission. In this way, the descriptions given recommendations for the treatment of AIHA may be equal to the evidence of D.

The first line of therapy.
Glucocorticosteroids.
The first line of therapy for AIHA patients with heat antibodies is glucocorticosteroids. The starting dose of prednisolone or metiprednisolone is 1 mg/kg (in the middle or intravenously). During 1-3 days of initial therapy (to be carried out in a hospital), the hematocrit level is greater than 30% or the hemoglobin value is greater than 100 g/l (there is no need to normalize the hemoglobin level). As the therapeutic meta has been reached, the dose of prednisolone is reduced to 20-30 mg per day for a long period of time. In case, if the numbers do not reach the end of the 3rd day, then the therapy of another line is connected. Dose reduction of prednisolone is continued at the outpatient stage. More dose reduction of prednisolone should be carried out at the time of reaching a therapeutic effect. Reduce the dose of prednisolone starting at 5-10 mg over 2-3 days and continue until the dose reaches 20-30 mg. Give the drug a significantly longer dose - 25 mg for 5-7 days. When reaching a dose lower than 10-15 mg, the rate of administration is due to more complications: 2.5 mg skin 2 times with the method of re-administration of the drug. This tactic transfers the validity of taking prednisolone for 3-4 months. Monitoring of hemoglobin, reticulocytes is carried out. In times, even if it lasts 3-4 months when taking prednisolone at a dose of 5 mg, a remission is obtained, you can try a new drug. Pragnennya quick dose change from the moment of normalization of hemoglobin through side activities GC (cushingoid, steroid drugs, arterial hypertension, eel visip with the molding of pustules on the skin, bacterial infections, diabetes mellitus, osteoporosis, venous thrombosis) may lead to relapse of hemolysis. Supportive therapy when treated with steroids may include bisphosphonates, vitamin D, calcium, which supports folic acid therapy. Monitoring of blood glucose level and active treatment of diabetes is carried out, because diabetes is a great factor in the risk of death through infection. It is necessary to assess the risks of thromboembolism legenian artery, especially in patients with AIHA and wolf anticoagulant or relapse of AIHA after splenectomy 38.

1st line glucocorticosteroid therapy is effective in 70-85% of patients; Prote more patients to save even hemoglobin in the range of 90-100 g / l require corticosteroid therapy, in 50% of patients the dose of 15 mg / dose is less and approximately 20-30% of patients in the required doses are higher. Note that corticosteroid monotherapy is less effective in 20% of patients. In patients with resistance to 1 line of therapy, it is necessary to re-evaluate the possibility of secondary AIHA, since AIHA with heat agglutinins is associated with malignant tumours, UC, teratoma of the ovaries and often with IgM.

Another line of therapy.
Splenectomy.
After splenectomy, there is a risk of important infections associated with Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae. Patients are given polyvalent pneumococcal, meningococcal, Haemophilus influenzae type b capsular polysaccharide (PRP) conjugated with right toxoid (TT) vaccine 2-4 days prior to splenectomy. In patients who received rituximab vaccination for the remaining 6 months, vaccination may be ineffective. After surgery - thromboprophylaxis with low doses of low molecular weight heparins; stepwise application of glucocorticoids according to the scheme described above, pneumococcal vaccine - skin 5 years. Patients after splenectomy should be informed about the risk of infection and the need for any febrile episode to take antibiotics from the penicillin group or respiratory fluoroquinolones (levofloxacin); also, the stench should be informed about the risk of venous thromboembolism.

Rituximab.


· Vіdmova vіd splenectomії;
· summer week with a high step, the risk of easing first and other line therapy
Contraindications before splenectomy, high risk of venous thromboembolism.


Active hepatitis B and C;

Standard Mode 375 mg/m2 on days 1, 8, 15 and 22. Patients who are on therapy with corticosteroids before starting rituximab therapy should continue taking corticosteroids until the first sign of rituximab acceptance.

Efficiencyü rituximab in a standard dose in AIHA with heat antibodies: acute response 83-87%, total response 54-60, relapse-free survival in 72% with a stretch of 1 year and 56% - a stretch of 2 years.
The hour of today varies from 1 month in 87.5% to 3 months - 12.5%. With a repeated course, the effectiveness of rituximab may be greater than with the first course. Consideration for therapy should be considered in monotherapy or in combination with corticosteroids, immunosuppressants and interferon-α and not in the primary therapy.

Toxicity of therapy: The drug has a garniy profile of safety. Even more rarely, as a rule, after the first infusion, fever, chills, visip, or at the throat. More serious reactions include erythromycosis I (rarely), bronchospasm, anaphylactic shock, thromboembolism of the legenous artery, thrombosis of the arteries of the intestine, infection (episodes of infection in about 7%), and development of fulminant hepatitis. leukoencephalopathy.
Rituximab at a low dose (100 mg/day for 4 days), like the first or other line of therapy, gave a general response in 89% (overall response in 67%) and a relapse-free period for a duration of 36 months in 68%. Approximately 70% of patients who were taking corticosteroids and rituximab in remission for 36 months were equal to 45% of patients who were taking steroid monotherapy.

Immunosuppressive drugs.
The main factor in choosing an immunosuppressive drug may be safe for the patient, so that the effectiveness of all drugs is considered low and may be unsafe for the patient, lower the risk of illness (Table 2). In case of trivial treatment, the therapy can be carried out at the outpatient stage under the supervision of a specialist.

Table 2 - AIHA immunosuppressive therapy

A drug	Dosing	Efficiency	Note
Azathioprine	100-150 mg/day or 1-2.5 mg/day for a trivalent period (4-6 months). Supportive therapy (25 mg every other day) may be given for 4 months up to 5-6 months.		Difficulty dosing through a therapeutic tube, hypersensitivity through genetic features or interaction with other drugs. Rarely manifested: weakness, thirst, elevated transaminases, severe neutropenia with infection, pancreatitis.
Cyclophosphamide	100 mg/day	Vidpovіd lesser, lower in 1/3 patients	In case of trivial jubilation, there is a mutagenic potential
Cyclosporin A		¾ of patients with AIHA with heat antibodies and life-threatening, refractory hemolysis 48 The combination of cyclosporine, prednisolone, and danazol gave an equal response in 89% of patients, compared to 58% of patients who switched to prednisolone and danazol therapy.	increased creatinine in blood serum, hypertension, increased stoma, paresthesia, hyperplasia clear, myalgia, dyspepsia, hypertrichosis, tremor
Mycophenolate mofetil	Pochatkova dose of 500 mg per dose with an increase of up to 1000 mg per dose for 2 to 13 months	Exchange of data at low rates in patients with refractory AIHA with heat antibodies. Successful blockage in patients treated with rituximab in multiple refractory AIHA after HSCT	head bіl, bіl across, abdominal distention, anorexia, nudota

Algorithm for emergency situations:
in case of suspicion of the presence of a hemolytic crisis (fever, pallor, yellowness of the skin, darkening of the sciatica, splenomegaly, cardiovascular insufficiency, anemic shock, anemic coma) - a call of the CMD team for emergency transport of the patient to hematology;
· monitoring of life-important functions: frequency and nature of breathing, frequency and rhythmicity of the pulse, signs of systolic and diastolic AT, number and color of the section;
· for obvious signs of impaired vital functions (heart failure, signs of shock, nirkovo insufficiency);

· doctor's consultation on X-ray endovascular diagnostics and treatment - insertion of a central venous catheter with peripheral access (PICC);
· consultation of a hepatologist - for the diagnosis and treatment of viral hepatitis;
· consultation of a gynecologist - in case of vaginess, metrorrhagia, menorrhagia, consultation in case of recognition of combination oral contraceptives;
· consultation of a dermatovenereologist – in case of skin syndrome;
consultation of an infectious disease specialist - in case of suspected viral infection;
· consultation of a cardiologist – in case of uncontrolled hypertension, chronic heart failure, disruption of heart rhythm and conduction;
· consultation of a neuropathologist – in case of acute cerebral hemorrhage, meningitis, encephalitis, neuroleukemia;
· consultation of a neurosurgeon – in case of acute cerebral hemorrhage, dislocation syndrome;
· Consultation of a nephrologist (eferentologist) – in case of a neurological deficiency;
· consultation of an oncologist - in case of suspicion of solid swelling;
· consultation of an otorhinolaryngologist - for the diagnosis and treatment of inflammation of the paranasal sinuses and the middle ear;
· consultation of an ophthalmologist - in case of damaged eyesight, inflammation of the eyes and appendages;
· consultation of a proctologist – in case of anal fissure, paraproctitis;
Psychiatric consultation - in case of psychoses;
· consultation of a psychologist – in case of depression, anorexia;
· Consultation of a resuscitator - in case of treatment of important sepsis, septic shock, acute lung injury syndrome in case of differentiation and terminal station syndrome, installation of central venous catheters.
consultation of a rheumatologist - with VKV;
· consultation of a thoracic surgeon - with exudative pleurisy, pneumothorax, zygomycosis legenia;
· consultation of a transfusiologist - for the selection of transfusion media in case of a positive indirect antiglobulin test, ineffective transfusion, acute massive hemolysis;
· consultation of a urologist – in case of infectious-flammable diseases of the sichoid system;
· consultation of a phthisiatrician – in case of suspected tuberculosis;
consultation of a surgeon - in case of surgical complications (infectious, hemorrhagic);
· Consultation of a splinter-facial surgeon - in case of infectious-flammable diseases of the dentition-slit system.

Preventive visit:
· In case of second AIGA-adequate treatment of the main illness;
· in case of AIHA with cold antibodies - unique hypothermia.

Monitoring will become a patient:
To monitor the effectiveness of the examination in the outpatient card, the following are indicated: the patient's fever, signs of the heart's blood test, including reticulocytes and platelets, biochemical indicators - rіven bilirubin, LDH, immunoenzyme test on the membrane.

Patient's individual care map

Category of patients	Global blood test, including reticulocytes	Biochemical analysis (bilirubin with fractions, LDH)	Direct Coombs test	Immunoenzymatic determination of the number of immunoglobulins on the membrane of erythrocytes	Hematologist consultation
Conservative exuberance	After reaching remission - 1 time per month;	The process of jubilation is not less than 1 time in 10 days; After reaching remission - 1 time for 2 months;	1 time for 3-6 months	1 time for 2 months	D obl_k that guarding at the hematologist for a living for 5 years.

Indicators of efficiency :
Criteria for eligibility
· Criteria for remission: improved hemogram indications (hemoglobin > 120 g/l, reticulocytes)< 20%), уровня непрямого билирубина и активности ЛДГ продолжительностью не менее 2 месяцев.
· Criteria for private remission: hemoglobin > 100 g/l, reticulocytes less than two norms, indirect bilirubin rate 25 µmol/l and lower for trivality not less than 2 months.
· Day of withdrawal for therapy ascertain for insignificant positive dynamics, or for a period of less than 1 month.

Likuvannya (stationary)

FUN IN THE STATIONARY RIVNI

Tactics of jubilation (UD-V): patients are hospitalized until hematological care, with impaired vital functions - at the resuscitation.

Non-drug treatment: diet for improving comorbidity, regimen - II.

Medical treatment:

1st line of therapy.

Glucocorticosteroids.
The first line of therapy for AIHA patients with heat antibodies is glucocorticosteroids. Corticosteroids, as a rule, prednisolone, are prescribed at a starting dose of 1 mg/kg per doba (50-80 mg/doba) for 1-3 days until an increase in hematocrit is greater than 30% or hemoglobin is greater than 100 g/l. In case, as the appointed meta is not reached by a stretch of 3 days, it may be different to another line of therapy, but the glucocorticoid therapy is considered to be ineffective. Increasing the dose of prednisolone up to 2 mg/kg/day (90-160 mg/day) does not change the results of treatment, but leads to a rapid development of characteristic important conditions. As the therapeutic meta has been reached, the dose of prednisolone is reduced to 20-30 mg per doba. Reduce the dose of prednisolone starting at 5-10 mg over 2-3 days and continue until the dose reaches 20-30 mg. Give the drug a significantly longer dose - 25 mg for 5-7 days. When reaching a dose lower than 10-15 mg, the rate of administration is due to more complications: 2.5 mg skin 2 times with the method of re-administration of the drug. This tactic transfers the validity of taking prednisolone for 3-4 months. Monitoring of hemoglobin, reticulocytes is carried out. In times, even if it lasts 3-4 months when taking prednisolone at a dose of 5 mg, a remission is obtained, you can try a new drug. Pragnennya to a sudden change in dose at the time of normalization of hemoglobin through side effects of GC (cushionoid, steroid viraz, arterial hypertension, acne visip with molding of abscesses on the skin, bacterial infection, thrombosis, osteoporosis). In fact, patients who take low doses of corticosteroids for more than 6 months may experience a lower relapse rate and a greater survival rate compared to patients who have been on therapy for up to 6 months of therapy.
An alternative to the trivial use of cortocosterosteroids (up to 3-4 months) is a short course (up to 3 days) to a course with an onset transition to another line of therapy.

In all patients who are switching to steroid therapy, they should take bisphosphonates, vitamin D, calcium, which should be supplemented with folic acid therapy. Monitoring of blood glucose level and active treatment of diabetes is carried out, because diabetes is a great factor in the risk of death through infection. It is necessary to evaluate the risk of thromboembolism of the legenous artery, especially in patients with AIHA and canine anticoagulant or relapse of AIHA after splenectomy.
Patients with particularly severe hemolysis and even severe anemia or folding depressions (Evans syndrome) should be administered methylprednisolone at a dose of 100-200 mg / day for 10-14 days or 250-1000 mg / day for 1-3 days. Therapy with high doses of corticosteroids in the literature is presented more importantly in view of the description of clinical manifestations. 19.20

1st line glucocorticosteroid therapy is effective in 70-85% of patients; Prote more patients to save even hemoglobin in the range of 90-100 g / l require corticosteroid therapy, in 50% of patients the dose of 15 mg / dose is less and approximately 20-30% of patients in the required doses are higher. Note that corticosteroid monotherapy is less effective in 20% of patients. In patients with resistance to 1 line of therapy, it is necessary to re-evaluate the possibility of secondary AIHA, in addition, AIHA with thermal agglutinins is associated with malignant tumors, UC, teratoma fraction of the ovaries and often with IgM.

Another line of therapy
When choosing a therapy, other lines are a few options, and for choosing a skin one, it is necessary to call measles/risk in different cases (Fig. 2).

Splenectomy.
Splenectomy, on a deep thought, is the most effective and appropriate method 2-line therapy for AIHA with heat antibodies.

Indications before splenectomy:
refractoriness or intolerance to corticosteroids;
· The need for post-treatment therapy with prednisolone at a dose of over 10 mg/dose;
· Partial recurrence.
Перевагами спленектомії є досить висока ефективність з досягненням часткової або повної ремісії у 2/3 пацієнтів (38-82% з урахуванням вторинних форм АІГА, при яких відповідь менша, ніж при ідіопатичній АІГА), значна кількість пацієнтів залишаються в ремісії, не вимагаючи медикаментозного втручання . stretching 2 years and more; the wearability is approximately 20%.
For splenectomy patients with persistent or recurrent hemolysis often require lower doses of corticosteroids, lower to splenectomy.

Insufficient splenectomy:
· Vіdsutnіst nadіynyh predictors of the result of splenectomy;
Risk of surgical complications (BODY, intracerebral bleeding, abscess of the empty cervix, hematoma) - 0.5-1.6% with laparoscopic splenectomy and 6% with primary splenectomy);
· Risk of infection - 3.3-5% (most safe pneumococcal septicemia) with mortality up to 50%.
After splenectomy, there is a risk of important infections associated with Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae. Patients are prescribed polyvalent pneumococcal, meningococcal, Haemophilus influenzae type b capsular polysaccharide (PRP) conjugated with prava toxoid (TT) vaccine 2-4 days before splenectomy. In patients who received rituximab vaccination for the remaining 6 months, vaccination may be ineffective.

After surgery - thromboprophylaxis with low doses of low molecular weight heparins; stepwise application of glucocorticoids according to the scheme described above, pneumococcal vaccine - skin 5 years. Patients after splenectomy should be informed about the risk of infection and the need for any febrile episode to take antibiotics from the penicillin group or respiratory fluoroquinolones (levofloxacin); also, the stench should be informed about the risk of venous thromboembolism.

Malyunok 2. Algorithm for the treatment of steroid-refractoryWAIHA.

Rituximab.
Indications for rituximab administration:
Resistant forms of AIHA with an increasing number of different conditions;
· Vіdmova vіd splenectomії;
· Summer age with a high level of risk to ease the first and other line therapy;
· Contraindications before splenectomy (massive obesity, technical problems), high risk of venous thromboembolism.

Contraindications prior to acceptance of rituximab:
drug intolerance;
Active hepatitis B and C;
acute viral and bacterial infection.

JubilationLast option” (therapy I see)
High-dose cyclophosphamide (50 mg/kg/day for 4 days) was effective in 5 out of 8 patients with refractory AIHA with thermal antibodies.
Alemtuzumab, having shown efficacy in the treatment of small groups of patients with refractory AIHA, prote, in association with high toxicity, is considered as the "last resort" in the treatment of important idiopathic AIHA, refractory to all.
Transplantation of hematopoietic stem cells. Information about HSCT in patients with AIHA with heat antibodies is surrounded by single depressions or small groups, mainly in Evans syndrome with reaching complete remission of approximately 60% in allogeneic and 50% in autologous TCM.

Supportive therapy.
Patients with AIHA may often require transfusion of erythrocyte mass with a method of maintaining a clinically acceptable level of hemoglobin, given before the effectiveness of specific therapy appears. Рішення про проведення трансфузії залежить не тільки від рівня гемоглобіну, але більшою мірою від клінічного статусу пацієнта та коморбідності (особливо ІХС, тяжких захворювань легень), їх загострення, швидкості розвитку анемії, наявності гемоглобінурії або гемоглобінеми та інших проявів тяжкого гемолізу.1 Пацієнту у критичної the clinical situation is not guilty, but it is caused by transfusion of erythrocyte mass, induces in depressions, if the presence of individual consciousness is revealed, the heat autoantibody shards are often panreactive. Еритроцитовмісні компоненти першої групи крові Rh-сумісні можуть бути безпечно призначені в екстрених випадках, якщо алоантитіла (зустрічаються у 12-40% пацієнтів з АІГА) обґрунтовано виключені на основі попереднього трансфузійного анамнезу та/або акушерського анамнезу (жінки, що не мають вагітностей та попередніх transfusions and people without a history of transfusions). In other patients, expansion of the phenotyping is carried out with the designation of Rh subgroup (C, c, E, e), Kell, Kidd, and S / s from the inoculation of monoclonal IgM antibodies and the selection of total erythrocyte mass for transfusion. In vignette vipadkas for the purpose of aloantithil vicory, methods of thermal autoadsorption or alogenous adsorption are used. At any time, a biological test was carried out.

Algorithm of AIGA matching with thermal antibodies of representations of a baby 3.
Figure 3. Algorithm for AIHA treatment with heat antibodies in mature adults




Jubilation of the second AIGA.
AIHA with heat antibodies in VKV.
Important therapy line 1 є steroids, the order of recognition is similar to the primary AIHA (table 3).

Table 3 - Secondary AIGA

Ill chi camp	1 line	2 line	After 2 lines	Last resort
Pervinna AIGA	Steroids	Splenectomy, rituximab	Azathioprine, mycophenolate mofetil, cyclosporine, cyclophosphamide	High dose cyclophosphamide, alemtuzumab
B- and T-cell non-Hodgkin's lymphoma	Steroids	Chemotherapy Rituximab (splenectomy for lymphoma with clitin marginal zone of the spleen)
Hodgkin's lymphoma	Steroids	Chemotherapy
Solid fluff	Steroids, surgical treatment
Dermoid cyst of the ovaries	Ovariectomy
GVH	Steroids	Azathioprine	Mofetil Mycophenolate	Rituximab, autologous bone marrow transplantation
Nonspecific virazkovy colitis	Steroids	Azathioprine		Total colectomy
Global Variable Immunodeficiency	Steroids, immunoglobulin G	Splenectomy
Autoimmune lymphoproliferative disease	Steroids	Mycophenolate mofetil	Sirolimus
Alogenous TCM	Steroids	Rituximab	Splenectomy, T-lymphocyte infusion
Organ transplantation (pidshlunkova lash)*	Consideration of immunosuppressive therapy, steroids	Splenectomy
Interferon alfa	Talking about interferon	Steroids
Pervinna Kholodova agglutinina ailment	Zahist in the form of hypothermia	Rituximab, chlorambucil		Eculizumab, bortezomib
Paroxysmal cold hemoglobinuria	Supportive therapy	Rituximab

Drug-induced AIHA with heat antibodies. At the present time, the most significant drug inductions of AIHA are those induced by drugs for the treatment of CLL, especially fludarabine. AIHA may develop on an hourly basis or after taking medications. AIHA, induced by fludarabine can be life threatening. AIHA is on steroids, but only ½ of the patients may go into remission. Other cases of AIHA symptoms with heat antibodies are associated with interferon-α therapy, especially in cases of hepatitis C.

Vedennya vaginosti at AIHA. Increased vagility and autoimmune hemolytic anemia are rare. Often there is a threat of interruption of vaginess. A piece-by-piece reversal of the vacancy of a large woman is not shown. Illness during the first hour of pregnancy in women with severe hemolytic crises and progressive anemia. Є warning of autoimmune hemolytic anemia, which is repeated, with skin new vaginess. In similar cases, it is recommended to change the vaginess and contraception. More important is the conservative tactics of conducting slopes. The main reason for treating autoimmune hemolytic anemia is glucocorticoid. In acute ailments, a high dose of prednisolone is required - 1-2 mg / kg per doba. The maximum dose of vagits is unacceptable, take 70-80 mg per day for short periods of time, protecting the interests of the fetus. About the effect of exuberance and the possibility of a rapid dose, one can judge by a drop in hemoglobin, a decrease in temperature, and a change in weakness. The dose of prednisolone is reduced step by step, gradually. During a crisis, the dose can be significantly lower: 20-30 mg / day. The lowering dose for an hour of vagity can be changed to 10-15 mg per doba, but take it as needed for a long period of vagity.
With severe ailments, transfusion therapy is often needed. However, blood transfusions are more likely to be associated with life-threatening indications (severe back pain, shock, hemoglobin drop to 30-40 g/l). Erythrocyte mass is selected by indirect Coombs test. Transfusion of erythrocytes is not the method of treating autoimmune hemolytic anemia, cerebrovascular disease.

With insufficiently effective medicinal treatment autoimmune hemolytic anemia caused by splenectomy with the method of removal of the head gland of antibody production. Splenectomy in this case is less effective, lower in case of congenital hemolytic anemia

In the case of secondary AIHA, the tactics of conducting pregnancy and the prognosis is rich in why to lie down in the main illness.

Perelik main medicinal products:

Glucocorticosteroids (the first line of AIHA therapy with heat antibodies):
Methylprednisolone, tablet, 16 mg;
· Methylprednisolone, for injections, 250 mg;
· Prednisolone, for injections 30 mg/ml 1 ml;
· Prednisolone, pigulka, 5 mg;

Monoclonal antibodies (another line of therapy):
rituximab;

Antisecretory drugs (supportive therapy for treatment with glucocorticosteroids):
Omeprazole;
Rabeprazole;

Immunosuppressors (another line of therapy):
azathioprine;
Cyclophosphamide;
Mycophenolate mofetil;
· Cyclosporine.

Perelik of additional medicinal products

Monoclonal antibodies (third line of therapy, therapy in a row):
Alemtuzumab;

Vaccines:
· Polyvalent pneumococcal vaccine.

Non-steroidal anti-seizure drugs:
· Paracetamol;
· Ketoprofen, for injections 100 mg/2 ml.

Antihistamines:
Chlorapiramine.

Antibacterial and antifungal drugs:
4th generation cephalosporin;
Amikacin;
Ciprofloxacin;
Levofloxacin;
· Meropenem;
Imipenem;
Fluconazole.

Antiviral drugs:
· aciclovir, cream for ovarian congestion;
acyclovir, pigulka, 400 mg;
· aciclovir, powder for preparation of infusions;
valaciclovir;
alganciclovir;
ganciclovir;
· Famciclovir.

Describe what needs to be done to correct the damage to the water, electrolyte and acid-base balance:
· Water for injections, rozchin for injections 5ml;
· Dextrose, for infusions 5% 250ml;
· Dextrose, for infusions 5% 500ml;
· potassium chloride, for intravenous administration 40 mg/ml, 10 ml;
· Mannitol, rozchin for injections 15%-200.0;
· Sodium chloride, for infusions 0.9% 500ml;
· Sodium chloride, for infusions 0.9% 250ml.

Antihypertensive drugs:
Amlodipine;
lisinopril;
· Nebivolol;
Captopril.

Antispasmodics:
Drotaverine.

Vasopressori:
Dopamine.

Antianemic drugs:
· Folic acid.

Blood components:
· Erythrocytic leukofiltrated mass.

Preparation table:
Transfer of medical care on outpatient and inpatient levels

№	A drug	Dosing	Trivality zastosuvannya	Riven evidence
Glucocorticosteroids
1	Prednisolone	administer at the starting dose - 1 mg/kg per doba (50-80 mg/dobu) for 1-3 days until the hematocrit is greater than 30% or the hemoglobin is greater than 100 g/l. In case, as the appointed meta is not reached by a stretch of 3 days, it may be different to another line of therapy, but the glucocorticoid therapy is considered to be ineffective. Increasing the dose of prednisolone up to 2 mg/kg/day (90-160 mg/day) does not change the results of treatment, but leads to a rapid development of characteristic important conditions. As the therapeutic meta has been reached, the dose of prednisolone is reduced to 20-30 mg per doba. Reduce the dose of prednisolone starting at 5-10 mg over 2-3 days and continue until the dose reaches 20-30 mg. Give the drug a significantly longer dose - 25 mg for 5-7 days. When reaching a dose lower than 10-15 mg, the rate of administration is due to more complications: 2.5 mg skin 2 times with the method of re-administration of the drug. This tactic transfers the validity of taking prednisolone for 3-4 months. Monitoring of hemoglobin, reticulocytes is carried out. In times, even if it lasts 3-4 months when taking prednisolone at a dose of 5 mg, a remission is obtained, you can try a new drug.	Variable. Up to 3-4 months and more at low doses	D
2	Methylprednisolone	Similar to prednisolone. It is also possible to use whiskers for pulse therapy at doses of 150-1000 mg 1-3 days intravenously.	Similar to prednisolone	D
Monoclonal antibodies
3	Rituximab	375 mg/m2 intravenously	on 1, 8, 15 and 22 days	D
4	Alemtuzumab	Internally for at least 2 years, 3 mg on the 1st day, 10 mg - on the 2nd day and 30 mg - on the 3rd day for good tolerance to the skin dose. Nadali is recommended until the dose becomes 30 mg per day 3 times per week. in one day.	The maximum trivality of lust becomes 12 tyzhniv.	D
Immunosuppressors
5	Azathioprine	100-150 mg/day or 1-2.5 mg/day for three periods	4-6 min. Supportive therapy (25 mg every other day) may be given for 4 months up to 5-6 months.	D
6	Cyclophosphamide	100 mg/day	Long-term under control OAC, OAM up to a total dose of 3-4 g	D
7	Cyclosporin A	5 mg/kg/day for 6 days, then up to 3 mg/kg/day	Long-term control of drug concentration	D
8	Mycophenolate mofetil	Pochatkova dose of 500 mg per doba with an increase of up to 1000 mg per doba	from 2 am to 13 pm	D

Іnshi vidi likuvannya: splenectomy (another line of therapy).

Indications for consultation of specialists: div. outpatient rіven.

Indications for transfer to intensive care and resuscitation:
signs of organ dysfunction;
· Damage to the vital functions, which is a direct threat to the life of the patient.

Indicators of the effectiveness of the treatment: div. outpatient rіven.

Away Vedennya- vytyag zі statsіonaru z recommendations podshoj lіkuvannya for mіsce zhіstvennya under the supervision of a hematologist and іnshih fahivtsіv (for the obviousness of the second AIHA, concomitant illnesses).

Hospitalization

Information

Dzherela and Literature

1. Minutes of the meeting of the Joint Commission on the quality of medical services of the MHSD RK, 2016
   1. 1) Clinical recommendations for the diagnosis and treatment of autoimmune hemolytic anemia / Ed. V.G. Savchenko, 2014.-26 p. 2) Hill Q., Stamps R., Massey E. and in. Guidelines on management of induced inmune and secondary autoimmune, haemolytic anaemia, 2012. 3) Lechner K, Jager U. How I treat autoimmune hemolytic anemias in adults. Blood. 2010; 16:1831–8. 4) Dussadee K, Taka O, Thedsawad A, Wanachiwanawin W. Incidence and risk factor relapses in idiopathic autoimmune hemolytic anemia. J Med Associate Thai. 2010; 93 (Suppl 1): S165-S170. 5) Lechner K, Jager U. How I treat autoimmune hemolytic anemias in adults. Blood. 2010; 16:1831–8. 6) Dierickx D, Verhoef G, Van Hoof A, Mineur P, Roest A, Triffet A, et al. Rituximab in autoimmune haemolytic anaemia and immune thrombocytopenic purpura: a Belgian retrospective multicentric study. J Intern Med. 2009; 266:484-91. 7) Maung SW, Leahy M, O'Leary HM, Khan I, Cahill MR, Gilligan O, et al. A multi-center retrospective study of rituximab that has been successful in recent relapsed or resistant warm hemolytic anemia. Br J Haematol. 2013;163:118–22. 8) Narat S, Gandla J, Hoffbrand AV, Hughes RG, Mehta AB. Rituximab in previous refractory autoimmune cytopenias in adults. haematologica. 2005; 90:1273-4. 9) Zanella A. et al. Treatment Of Autoimmune Hemolytic Anemias, Haematologica October 2014 99: 1547-1554. 10) Peñalver FJ, Alvarez-Larran A, Diez-Martin JL, Gallur L, Jarque I, Caballero D, et al. Rituximab is an effective and safe therapeutic route in adults with a refractor and severe autoimmune hemolytic anemia. Ann Hematol. 2010; 89:1073-80. 11) O Connell N, Goodyer M, Gleeson M, Storey L, Williams M, Cotter M, et al. Confirmation of treatment with rituximab and mycophenolate morphef with refractor autoimmune hemolytic anemia post-hematopoietic stem cell transplant for dyskeratosis congenita due to TINF2 mutation. Pediatric Transplant. 2014;18(1):E22–24. 12) Dussadee K, Taka O, Thedsawad A, Wanachiwanawin W. . Incidence and risk factors of relapses in idiopathic autoimmune hemolytic anemia. J Med Associate Thai. 2010; 93 (Suppl 1): S165-S170. 13) Lechner K, Jager U. How I treat autoimmune hemolytic anemias in adults. Blood. 2010; 16:1831–8. 14) Murphy S, LoBuglio AF. Drug therapy of autoim-mune hemolytic anemia. Semin Hematol. 1976; 15) Gehrs BC, Friedberg RC. Autoimmune hemolytic anemia. Am J Hematol. 2002; 69(4): 258-271. 16) Akpek G, McAneny D, 3. Weintraub L. Akpek G, McAneny D, Weintraub L Comparative response to splenectomy in Coombs-positive auto-immune hemolytic anemia in no time. Am J Hematol. 1999; 61:98-102. 17) Casaccia M, Torelli P, Squarcia S, Sormani MP, Savelli A, Troilo BM, etc. Laparoscopic splenectomy for hematological diseases: a forward analysis of vicons at the Italian Registry Laparoscopic Surgery of the Spleen (IRLSS). Surg Endosc. 2006; 20:1214–20. 18) Bisharat N, Omari H, Lavi I, Raz R. Risk of infection and death among post-splenectomy patients. J Infect. 2001; 43:182-6. 19) Davidson RN, Wall RA. Prevention and management of infections in patients without spleen. Clin Microbiol Infect. 2001; 7:657–60. 20) Borthakur G, O'Brien S, Wierda WG, et al. Immune anaemias in pacients with chronic lympho-cytic leukaemia treated with fludarabine, cyclo-phosphamide and rituximab–incidence and predictors. Br J Haematol. 2007; 136(6): 800-805. 21) Chiao EY, Engels EA, Kramer JR, et al. Risk of immune thrombocytopenic purpura and autoimmune hemolytic anemia among 120,908 US vet-erans with hepatitis C virus infection. Arch Intern. Med. 2009; 169(4): 357-363.

Information

SHORT THINGS TO BE VICTORATED BY THE PROTOCOL:

AT -	arterial vice
AIGA -	autoimmune hemolytic anemia
ALT -	alanine aminotransferase
AST -	alanine aminotransferase
VIL -	human immunodeficiency virus
GGTP -	gammaglutamyl transpeptidase
IFA -	enzyme immunoassay
CT -	Computed tomography
LDH -	Lactate dehydrogenase
INR -	international normalization
MRI -	Magnetic resonance imaging
NHL -	non-Hodgkin's dymphomi
UAC -	deep blood test
OAM -	hot analysis of the section
UAR -	operational and anesthetic risk
PNG -	paroxysmal night hemoglobinuria
PTI -	prothrombin index
CPR -	heart-legenev resuscitation
SMD -	Svidka medical aid
TKM -	transplantation of the cystic brain
TSH -	thrombotic thrombocytopenic purpura
UHF -	jets of ultrahigh frequency
UZDG -	ultrasound dopplerography
Ultrasound -	ultrasonic follow-up
BH -	breathing frequency
Heart rate -	heart rate
CNS -	central nervous system
HLL -	chronic lymphocytic leukemia
FGDS -	fibrogastroduodenoscopy
ECG -	electrocardiography
MRI -	nuclear magnetic resonance imaging
CAIHA-	Autoimmune hemolytic anemia with cold antibodies
cd-	cluster of differentiation
DAT-	Direct Coombs test
Hb-	hemoglobin
Ht -	hematocrit
WAIHA-	Autoimmune hemolytic anemia with heat antibodies

List of retailers in the protocol from the assigned qualification data:
1) Turgunova Lyudmila Gennadiivna - Doctor of Medical Sciences, Professor of the RDP at the REM "Karaganda State Medical University", Head of the Department of Therapeutic Disciplines, Faculty of Continuous Professional Development, Hematologist.
2) Pivovarova Irina Oleksiivna – MD MBA, Head of the RGO “Kazakhstan Association of Hematology Doctors”, auditor of TOV “Center of Hematology”.
3) Klodzinsky Anton Anatoliyovich - Candidate of Medical Sciences, hematologist at TOV "Center of Hematology".
4) Khan Oleg Ramualdovich - Assistant of the Department of Therapy postgraduate education, doctor-hematologist (RSE on REM NDI cardiology and internal ailments).
5) Satbaeva Elmira Marativna - Candidate of Medical Sciences, RDP at REM "Kazakh National Medical University named after. S.D. Asfendiyarov”, Head of the Department of Pharmacology.

Statement on the day of the conflict of interest: no.

List of reviewers:
1) Ramazanova Raigul Mukhambetivna - Doctor of Medical Sciences, head of the course of hematology, AT "Kazakh Medical University of Continuous Education".

Addendum 1

List of drugs that may cause autoimmune hemolysis or lead to the detection of antierythrocytic antibodies

No. p / p	International non-proprietary name
1.	Acetaminophen
2.	Acyclovir
3.	Amoxicillin
4.	Amphotericin B
5.	Ampicillin
6.	Acetylsalicylic acid
7.	Carbimazole
8.	Carboplatin
9.	Cefazolin
10.	Cefixim
11.	Cefotaxime
12.	cefotetan
13.	Cefoxitin
14.	Cefpir
15.	Ceftazidime
16.	Cefuroxime
17.	Chloramphenicol
18.	Chlorpromazine
19.	Ciprofloxacin
20.	Cisplatin
21.	Diclofenac
22.	Cedolac
23.	Ethambutol
24.	Fenoprofen
25.	Fluconazole
26.	Hydralazine
27.	Ibuprofen
28.	Imatinib
29.	insulin
30.	Isoniazid
31.	Ofloxacin
32.	Melphalan
33.	Mercaptopurine
34.	Methotrexate
35.	Naproxen
36.	Norfloxacin
37.	Oksaliplatin
38.	Piperacillin
39.	Ranitidine
40.	Streptokinase
41.	Streptomycin
42.	Sulfasalazine
43.	Sulindak
44.	Tetracycline
45.	Ticarcilin
46.	Thiopental sodium
47.	Co-trimoxazole
48.	Vancomycin
49.	fludarabine
50.	Cladribine

Attached files

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What is autoimmune hemolytic anemia

Under the name of hemolytic anemia, there is a group of swelling and recessive diseases that are characterized by advancing intracellular or intravascular erythrocyte collapse.

Autoimmune hemolytic anemia include forms of illness, associated with antibodies to erythrocyte body antigens.

In the global group of hemolytic anemias, autoimmune hemolytic anemias are most common. Their frequency becomes 1 per 75000-80000 population.

Causes (etiology) of autoimmune hemolytic anemia

Immune hemolytic anemia can be attributed to the influx of anti-erythrocyte iso- and autoantibodies, apparently, subsuming on iso-immune and auto-immune.

Hemolytic anemias of newborns are seen to be immunocompetent, there is no confusion in the ABO systems and Rhesus between mother and fetus, post-transfusion hemolytic anemias.

In case of autoimmune hemolytic anemia, there is a prospect of immunological tolerance to unchanged antigens in blood erythrocytes, and sometimes - to antigens, which may be similar to erythrocytes of determinants. Antibodies to similar antigens can intermingle with the same antigens of wet erythrocytes. Non-thermal agglutination is the most common variety of antibodies, common causes of development of autoimmune hemolytic anemia. Antibody counts up to IgG, rarely - up to IgM, IgA.

Immune hemolytic anemias are subdivided into isoimmune and autoimmune. The serological principle of differentiation of autoimmune hemolytic anemias allows for the appearance of different forms of heat agglutinins, thermal hemolysins, cold agglutinins, biphasic cold hemolysins (Donat-Landsteiner type) and erythroopsonins. The authors see a form of hemolytic anemia with antibodies against the antigen of normoblasts of the cystic brain.

per clinical break see more and more chronic options.

Distinguish symptomatic and idiopathic autoimmune hemolytic anemia. Symptomatic autoimmune anemia blame on the background small illnesses, which are accompanied by disorders in the immune system. Most often, stinks are associated with chronic lymphocytic leukemia, lymphogranulomatosis, acute leukemia, systemic vermicompost, rheumatoid arthritis, chronic hepatitis and cirrhosis of the liver. In quiet situations, if the appearance of autoantibodies does not seem to be associated with any pathological process, we can talk about idiopathic autoimmune hemolytic anemia, as it becomes close to 50% of all autoimmune anemia.

The autoantibody releases are later damaged in the system of immunocompetent cells, so they accept the erythrocyte antigen as a foreign one and repair it to a new antibody. After fixation of autoantibodies on erythrocytes, the remainder are engulfed by clitins of the reticulo-stiocytic system, de-recognizing agglutination and decay. Erythrocytic hemolysis is the leading rank in the spleen, liver, and cystic brain. Autoantibodies to erythrocytes lie down to different types.

According to the serological principle, autoimmune hemolytic anemia is subdivided into sprat forms:
- anemia with incompetent heat agglutinins
- anemia with thermal hemolysins
- anemia with severe cold agglutinins
- anemia with biphasic hemolysins
- anemia with agglutinins against normoblasts of the cystic brain

The skin of these forms may have specific features in the clinical picture, escaping and serological diagnosis. Anemia with non-incomplete heat agglutinins is most common, which accounts for 70-80% of all autoimmune hemolytic anemias.

Pathogenesis

The significance of autoimmune processes lies in the fact that, as a result of the weakening of the T-suppressor system, the immune system, which controls autoaggression, activates the B-system of the immune system, which is synthesized with this antibody against unaltered antigens in various organs. The implementation of autoaggression also takes the fate of T-lymphocyte-killers. Antitila - all immunoglobulins (Ig), which lie most often up to class G, lower - M and A; stench is specific and directed against antigen. Before IgM, zocrema, cold antibodies and biphasic hemolysins are seen. The erythrocyte, which carries antibodies, is phagocytized by macrophages and collapses in them; possible lysis of erythrocytes for the participation of complement. Antibodies to the IgM class can cause agglutination of erythrocytes directly in the bloodstream, and antibodies to the IgG class can cause damage to erythrocytes only in spleen macrophages. In all types of hemolysis of erythrocytes, it is more intense, the more antibodies are found on their surface. Hemolytic anemia with antibodies to spectrin has been described.

Symptoms ( clinical picture) autoimmune hemolytic anemia

With acute onset of autoimmune hemolytic anemia, patients develop rapidly increasing weakness, back and heartbeat, pain at the heart, sometimes across, increased body temperature and vomit, intense zhovtyanitsya. In case of chronic interruption of the process, it is evidently self-consciously ill with severe anemia, often manifested zhovtyanitsa, in most cases there is an increase in the spleen, sometimes liver, sever periods of acute and remitting.

Anemia may be normochromic, sometimes hyperchromic in nature, with hemolytic crises, there are signs of expressions or reticulocytosis subsides. In peripheral blood, macrocytosis and microspherocytosis of erythrocytes are observed, normoblasts may appear. SHOE in more vipadkіv zbіlsheno. The number of leukocytes in the chronic form becomes normal, in the acute form - leukocytosis develops, which sometimes reaches high numbers with significant damage to the leukocyte formula to the left. The platelet count is normal.

In Fischer-Ivens syndrome, autoimmune hemolytic anemia is combined with autoimmune thrombocytopenia. In the cystic brain, erythroposis of settlements, megaloblasts are rarely seen. In most patients, the osmotic resistance of erythrocytes is reduced, which is indicated by a significant number of microspherocytes in peripheral blood. Instead of bilirubin, it was increased for the rahunok of the free fraction, it was promoted instead of stercobilin in kali.

Inadequate thermal agglutination is detected by the help of a direct Coombs test with polyvalent sulfurate. In case of a positive test for additional anti-antibodies to IgG, IgM, etc., it is specified to which class of immunoglobulins the antibodies that are detected are included. Although there are less than 500 fixed IgG molecules on the surface of erythrocytes, the Coombs test was negative. A similar phenomenon may be seen in patients with a chronic form of autoimmune hemolytic anemia, or have suffered hostile hemolysis. Coombs-negative ivyavlyayutsya and depressions, if on erythrocytes fixed antibodies, scho lie up to IgA or IgM (according to the ratio to which polyvalent antiglobulin sirovatka is less active).
Approximately 50% of patients with idiopathic autoimmune hemolytic anemias, one hour with the appearance of immunoglobulins, fixed on the surface of erythrocytes, show antibodies to salivary lymphocytes.

Hemolytic anemia, induced by thermal hemolysins, rare. It is characterized by hemoglobinuria with visions of a black color, blackening of periods of acute hemolytic crisis and remissions. Hemolytic crisis is accompanied by development of anemia, reticulocytosis (in other cases thrombocytosis) and enlargement of the spleen. An increase in the free fraction of bilirubin, hemosiderinuria is indicated. When examining donor erythrocytes with papain, it can be detected in patients with monophasic hemolysin. Some patients have a positive Coombs test.

Hemolytic anemia, aggravated by cold agglutinins(cold hemagglutinin ailment) may be chronic. It develops with a sharp increase in the titer of cold hemagglutinins. Distinguish between idiopathic and symptomatic forms of illness. The leading symptom of the disease is supernaturally elevated sensitivity to the cold, as manifested by the blue and white fingers of the hands and the lower, wuh, and tip of the nose. Disorders of peripheral blood circulation lead to the development of Raynaud's syndrome, thrombophlebitis, thrombosis and trophic changes right up to acrogangrene, sometimes cold bloody kropivyanki. The culprit of vasomotor disorders is associated with closures in case of cooling of the great intravascular conglomerates from agglutinated erythrocytes with onset spasm of the vessel wall. Qi changes will increase with more importantly internal cellular hemolysis. Some of the ailments have an enlarged liver and spleen. There are moderately pronounced normochromic or hyperchromic anemia, reticulocytosis, normal number of leukocytes and platelets, an increase in SOE, a slight increase in the level of the free fraction of bilirubin, a high titer of cold agglutination is detected by the method of salt glutination. The agglutination of erythrocytes in vitro is typical, which is due to room temperature and occurs when heated. If immunological tests are not possible, the diagnostic value of a provocative test for colds is increased (in the case of blood sputum, cut off from a finger tied with a tourniquet after lowering it into the water, it is indicated as a movement in the place of free hemoglobin).

In case of cold hemagglutinin ailment in the presence of paroxysmal cold hemoglobinuria, the hemolytic crisis and vasomotor impairment are blamed only on hypothermia of the body and hemoglobinuria, which began in the minds of cold, is due to the transition of ailment to heat.

The symptom complex, the dominant cold hemagglutinin disease, can be blamed on aphids of various host infections and other forms of hemoblastosis. In case of idiopathic forms of illness, there is no recurrence, in case of symptomatic prognosis, it is the main rank of severity of the main process.

Paroxysmal cold hemoglobinuria can lead to rare forms of hemolytic anemia. On it, people of both articles, often children, get sick.

In ailments with paroxysmal cold hemoglobinuria, after a change in the cold, severe ailments, headaches, body aches and others may appear. unacceptable sight. After that, chills begin, the temperature rises, boredom and vomit are indicated. The slaughter is full of black zabarvlennya. At the same time, yellowness, enlargement of the spleen and vasomotor damage appear at times. On aphids of a hemolytic crisis, ailments show a dead anemia, reticulocytosis, an increase in the free fraction of bilirubin, hemosiderinuria and proteinuria.

Residual diagnosis of paroxysmal cold hemoglobinuria is established on the basis of detection of biphasic hemolysins by the Donat-Landsteiner method. For her, autoagglutination of erythrocytes is not typical, which is constantly guarded against cold hemagglutination of the disease.

Hemolytic anemia, induced by erythroopsonins. Iznuvannya autoopsoniniv to blood cells є zagaloviznim. The phenomenon of autoerythrophagocytosis was revealed in case of nabutiy idiopathic hemolytic anemia, liver cirrhosis, hypoplastic anemia with hemolytic component and leukemia.

Nabuta idiopathic hemolytic anemia, which is accompanied by a positive phenomenon of autoerythrophagocytosis, may be chronic. The period of remission, which is sometimes a three-hour period, is changed by a hemolytic crisis, which is characterized by icterism of visible mucous membranes, darkening of the cross section, anemia, reticulocytosis and advancing of the indirect fraction of the liver, liver.

In idiopathic and symptomatic hemolytic anemias, the manifestation of autoerythrophagocytosis for the presence of data, which indicates the presence of other forms of autoimmune hemolytic anemias, may lead to the diagnosis of hemolytic anemia, it is reasonable. A diagnostic test for autoerythrophagocytosis is carried out in direct and indirect variants.

Immune hemolytic anemia, susceptibility to antibiotics. Various drugs (quinine, dopegyt, sulfanilamide, tetracycline, zeporin and others), virucidal hemolysis, dissolve complexes with specific heteroantibodies, then settle on erythrocytes and add complement to itself, which causes the membranes to break. Such a mechanism of drug-induced hemolytic anemia is confirmed by the presence of immunoglobulins on the erythrocytes of patients with complement. Anemias are characterized by host cob with signs of intravascular hemolysis (hemoglobinuria, reticulocytosis, substitution of the free fraction of bilirubin, exacerbation of erythropoiesis). On aphids of a hemolytic crisis, hospital deficiency sometimes develops.

Sometimes hemolytic anemias develop in a different way, which develops with recognition of penicillin and methyldopa. The introduction of more than 15,000 OD of penicillin can lead to the development of hemolytic anemia, which is characterized by intracellular hyperhemolysis. Some of the most important clinical and laboratory signs of hemolytic syndrome are also positive direct Coombs' test (antibodies, which are detected, are found up to IgG). Penicillin, binding to the antigen of the erythrocyte membrane, creates a complex against which antibodies are vibrated in the body.

In case of trivial methyldopa stagnation, some of the ailments cause hemolytic syndrome, which may cause idiopathic forms of autoimmune hemolytic anemia. The antibodies that appear are identical to thermal agglutinins and are elevated to IgG.

Hemolytic anemia, conditioned by mechanical factors, is associated with the collapse of erythrocytes during their passage through changes in the vessel or valve valves. The endothelial vessel changes with vasculitis, malignant arterial hypertension; with which adhesion and aggregation of platelets are activated, as well as the system of blood glottis and thrombin release. Expanding blood stasis and thrombosis of other blood vessels (DIC) with traumatization of erythrocytes develop, after which the stench is fragmented; in a blood smear, there are numerical fragments of erythrocytes (schistocytes). Ruinyuyuyutsya erythrocytes also for their passage through the piece valves (often - with multi-valve correction); described hemolytic anemia in the background of senile calcified aortic valve. Diagnosis is based on signs of anemia, increased concentration of free bilirubin in the blood sputum, presence of schistocytes in the peripheral blood smear, and symptoms of underlying disease that caused mechanical hemolysis.

Hemolytic-uremic syndrome(Moshkovich's disease, Gasser's syndrome) may aggravate the overcoming of autoimmune hemolytic anemia. Illness of an autoimmune nature is characterized by hemolytic anemia, thrombocytopenia, and urticaria. Disseminated damage to the vessels and capillaries from radiation of almost all organs and systems, changes in the side with coagulograms, characteristic of DIC, are indicated.

Diagnosis of autoimmune hemolytic anemia

Diagnosis of autoimmune hemolytic anemia is based on the presence of clinical and hematological signs of hemolysis and the presence of autoantibodies on the surface of erythrocytes for an additional Coombs test (60% of autoimmune hemolysis is positive). Differentiation of disease due to recessive microspherocytosis, hemolytic anemia associated with enzyme deficiency.

In the blood - normochromic or moderately hyperchromic anemia of various virulence, reticulocytosis, normoblasts. In a number of cases, blood smears show microspherocytes. The number of leukocytes may increase during a hemolytic crisis. The number of thrombocytes, as a rule, is within the limits of the norm, but thrombocytopenia may increase. SHOE is significantly reduced. In the cystic brain, there is a pronounced hyperplasia of the erythroid pariet. In the amount of bilirubin in the blood, as a rule, the movements for the chest are indirect.

Treatment of autoimmune hemolytic anemia

In acute forms of advanced autoimmune hemolytic anemia, prednisolone is prescribed at an additional dose of 60-80 mg. If ineffective, may be increased to 150 mg and more. The additional dose of the drug is divided into 3 parts at a ratio of 3:2:1. In the world of subsiding hemolytic crisis, the dose of prednisolone is progressively reduced (2.5-5 mg per day) to half the time. Further lowering the dose of the drug, in order to avoid the recurrence of the hemolytic crisis, is reduced by 2.5 mg for 4-5 days, then in smaller doses and at longer intervals until the drug is re-administered. In chronic autoimmune hemolytic anemia, it is enough to recommend 20-25 mg of prednisolone, and in the world I'll become a stalker sick and indicative of erythropoiesis, transfer to an intermediate dose (5-10 mg). In case of cold hemagglutinin disease, similar therapy with prednisolone is indicated.

Splenectomy in autoimmune hemolytic anemia associated with thermal agglutinins and autoerithroopsonins may be recommended only in patients with ailments, in which corticosteroid therapy is accompanied by non-trial remissions (up to 6-7 months) or resistance to є. In patients with hemolytic anemia, slurred with hemolysins, splenectomy does not prevent hemolytic crises. However, the stench is guarded earlier, lower before surgery, and it is easier to stop with additional corticosteroid hormones.

In refractory autoimmune hemolytic anemia, patients treated with prednisolone may be treated with immunosuppressants (6-mercaptopurine, imuran, chlorbutine, methotrexate, cyclophosphamide, etc.).

At the stage of a deep hemolytic crisis, a transfusion of erythrocyte mass should be performed, which is used for the help of an indirect Coombs test; to reduce the severity of endogenous intoxication, gemodez, polydez and other detoxification diseases are prescribed.

Treatment of hemolytic-uremic syndrome, which can aggravate the overcoming of autoimmune hemolytic anemia, including corticosteroid hormones, fresh frozen plasma, plasmapheresis, hemodialysis, transfusion or cryopreservation of erythrocytes. Regardless of the complex of current therapeutic benefits, the prognosis is often unfavorable.

It is up to some doctors to tell if you have autoimmune hemolytic anemia.

Hematologist

KID according to ICD-10

D59.1 Other autoimmune hemolytic anemias