Immunohemolitic anemia

Description:

Immunohemolitic anemias — group of the anemias relating to immunodependent ekzoeritrotsitarny hemolitic anemias. Etiolgiya of these anemias is connected with induction of an autoallergy to own erythrocyte antigens, an immune response on the haptens and antigens adsorbed by erythrocytes, immunocomplex reactions or with an alloimmunization.

Symptoms of Immunohemolitic anemia:

At the acute beginning of autoimmune hemolitic anemias patients have quickly increasing weakness, an asthma and heartbeat, pains in heart, sometimes in a waist, fervescence and vomiting, intensive jaundice. At the chronic course of process note rather satisfactory health of patients even at deep anemia, quite often expressed jaundice, in most cases increase in a spleen, sometimes and a liver, alternation of the periods of an aggravation and remission.
Anemia carries normokhromny, sometimes hyperchromic character, at hemolitic crises the expressed or moderate reticulocytosis is usually noted. In peripheral blood the macrocytosis and a microspherocytosis of erythrocytes is found, emergence of normoblasts is possible. SOE is in most cases increased. The maintenance of leukocytes at a chronic form happens normal, at acute — the leukocytosis reaching sometimes high figures with a considerable deviation to the left meets. Quantity of thrombocytes usually normal.
At Fischer's syndrome — Ivensa autoimmune hemolitic anemia is combined with autoimmune thrombocytopenia. In marrow эритропозз it is strengthened, megaloblasts seldom come to light. At most of patients osmotic resistance of erythrocytes is reduced that is caused by considerable number of microspherocytes in peripheral blood. Content of bilirubin is increased at the expense of free fraction, also the content of stercobilin in Calais is increased.
Incomplete thermal agglutinins are found by means of direct test of Koombs with polyvalent antiglobulinovy serum. At positive test by means of antiserums to IgG, IgM it is etc. specified to what class of immunoglobulins the revealed antibodies belong. If on a surface of erythrocytes less than 500 fixed molecules IgG, Koombs's test it is negative. The similar phenomenon is observed usually at patients with a chronic form of autoimmune hemolitic anemia or transferred acute hemolysis. Koombs-negative there are also cases when on erythrocytes the antibodies belonging to IgA or IgM are fixed (concerning which polyvalent antiglobulinovy serum is less active).
Approximately in 50% of cases of idiopathic autoimmune hemolitic anemias along with emergence of the immunoglobulins fixed on a surface of erythrocytes antibodies to own lymphocytes come to light.

Reasons of Immunohemolitic anemia:

Erythrocytes of patients at immune hemolysis collapse:
1. Owing to linkng of their native antigens with antibodies and, in the subsequent — the complement components which are carrying out a direct cytolysis — an autoimmune complement-mediated hemolysis.
2. On the similar mechanism with the opsonizing action of autoantibodies and disintegration of fagotsitirovanny erythrocytes — an autoimmune intracellular hemolysis
3. Owing to adsorption of cell-bound immune complexes with participation of not erythrocyte antigens and the subsequent a complement - a dependent lysis — "the mechanism of the innocent witness".
It can be observed at a system lupus erythematosus, malignant new growths, a leukosis, a limfogranulomatoza.

Treatment of Immunohemolitic anemia:

At acute forms the acquired autoimmune hemolitic anemias appoint Prednisolonum in a daily dose of 60 — 80 mg. At inefficiency it can be increased to 150 mg and more. The daily dose of drug is divided into 3 parts in the ratio 3:2:1. In process of subsiding of hemolitic crisis Prednisolonum dose gradually decreases (on 2,5 — 5 mg a day) to a half initial. The further dose decline of drug in order to avoid recuring of hemolitic crisis is carried out on 2,5 mg within 4 — 5 days, then in smaller doses and with big intervals before full drug withdrawal. At chronic autoimmune hemolitic anemia it is enough to appoint 20 — 25 mg of Prednisolonum, and in process of improvement of the general condition of the patient and indicators of an erythrogenesis to transfer to a maintenance dose (5 — 10 mg). At a cold gemagglyutininovy disease similar therapy is shown by Prednisolonum.
The splenectomy at autoimmune hemolitic anemia, connected with thermal agglutinins and autoeritroopsonina, can be recommended only to patients at whom corticosteroid therapy is followed by short remissions (up to 6 — 7 months) or there is a resistance to it. At patients with the hemolitic anemia caused by hemolysins, the splenectomy does not prevent hemolitic crises. However they are observed less than before operation, and are easier stopped by means of corticosteroid hormones.
At refractory autoimmune hemolitic anemias in combination with Prednisolonum immunodepressata can be used (6 Mercaptopurinum, an imuran, Chlorbutinum, a methotrexate, cyclophosphamide, etc.).
In a stage of deep hemolitic crisis apply transfusions of the eritrotsitny weight which is picked up by means of indirect test of Koombs; for decrease in the expressed endogenous intoxication appoint Haemodesum, Polydesum and other disintoxication means.
Treatment of a gemolitiko-uraemic syndrome which can complicate the course of autoimmune hemolitic anemias includes corticosteroid hormones, freshly frozen plasma, a plasma exchange, a hemodialysis, transfusions of the washed or cryotinned erythrocytes. Despite use of a complex of modern therapeutic means, the forecast often adverse.