FYShERA — EVANS the SYNDROME
(J. A. Fisher; R. S. Evans, sovr. amer. doctor; syndrome; a synonym Evans's syndrome) — a combination of autoimmune hemolitic anemia to autoimmune thrombocytopenia.
The disease was described for the first time by Fischer in 1947 and assumed immune genesis of anemia and thrombocytopenia. Evans and sotr. in 1951 described clinic of the autoimmune hemolitic anemia (see) which is combined with autoimmune thrombocytopenia (see) in more detail. Allocate a symptomatic and idiopathic form F. — AA. page.
The symptomatic form is observed at hron. hepatitis, system lupus erythematosus, hron. lymphoid leukosis, lymphoma, sarcoma, lymphogranulomatosis, pseudorheumatism, tuberculosis, etc. In rare instances the syndrome is the first display of these diseases. At an idiopathic form to establish connection of hemolysis and thrombocytopenia with any other patol. process does not work well.
The increased destruction of erythrocytes and thrombocytes owing to fixing on their surface of proteins of the immunoglobin nature — autoantibodies is the cornerstone of a pathogeny (see). Anti-erythrocyte antibodies more often are incomplete thermal agglutinins (see Agglutination) and belong to immunoglobulins (see) various classes (G, is more rare — M or And). They specifically contact antigens of system a Rhesus factor (see Blood groups); in nek-ry cases are directed against antigens of other systems. Specificity of an anti-thrombocyte of rny
immunoglobulin of a class G (antibodies) is not established, however is proved that its contents in comparison with norm is increased by surfaces of erythrocytes. Destruction of erythrocytes and thrombocytes is carried out preferential in a spleen, sometimes in a liver and marrow.
In this regard the producing the hemopoietic cells in marrow is increased, in a miyelogramma (see) increase in maintenance of erythroidal cells and megacaryocytes is noted (see the Hemopoiesis).
The wedge, a picture is characterized by the anemia, a high reticulocytosis and an indirect hyperbilirubinemia which are combined with thrombocytopenia, i.e. the symptoms inherent to autoimmune hemolitic anemia (see) and a Werlhof's disease (see the Purpura thrombocyte-penichesky). More often the disease begins gradually; patients complain of joint pains, a stomach, subfebril-ny temperature. Later moderate weakness and odyp joins, on skin hemorrhages (ecchymomas, petechias), hemorrhages in a mucous membrane of a mouth, a conjunctiva are found, nasal and uterine bleedings are observed. The hemorrhagic syndrome (internal N outside bleedings, hemorrhages) can precede emergence a wedge, and a lab. signs of immune hemolysis (see). In nek-ry cases hemolysis begins earlier. At a number of patients anemia and thrombocytopenia come to light at the same time. Thrombocytopenia can develop in several years after the splenectomy (see) executed concerning autoimmune hemolitic anemia, at the same time signs of hemolysis can be absent.
The diagnosis is established on the basis by a wedge, pictures and the direct test of Koombs (see Koombs reaction) confirming the autoimmune nature of hemolysis. Negative takes of test of Koombs do not exclude existence at sick immune hemolysis since during the strengthening of hemolysis a considerable part of the erythrocytes loaded with antibodies collapses. Also detection of the increased content in blood of reticulocytes, shortenings
of life expectancy of erythrocytes, increases in cells of an erythroidal row in marrow helps to establish hemolysis. At detection of thrombocytopenia and raised (or normal) quantities of megacaryocytes in marrow diagnose trombotsi-toliz (process of disintegration of thrombocytes). Immune character of a trombotsntope-niya confirms the quantitative method of definition of immunoglobulins of a class G offered by R. Dixon and W. Rosse on a surface of thrombocytes; at F. — AA. the page their contents on a surface of thrombocytes is raised.
Treatment is carried out by glucocorticoid hormones; in the absence of effect produce about captivity ek t of ohms and yu (see). If after operation the expressed hemolysis and (or) trombotsitoliz is recured, appoint tsitostati-
chesky drugs (Azathioprinum, Cyclophosphanum, Vincristinum), usually they are combined with glucocorticoid hormones. Children in the absence of vital indications of purpose of cytostatic drugs should avoid. At heavy anemia, life-threatening the patient, transfusions of eritro-tsitny weight or repeatedly washed erythrocytes which are picked up individually by means of indirect test of Koombs are shown. Transfusion of trom-botsitny weight does not render effect since donor cells quickly collapse.
The forecast is often adverse. At F. — AA. pages are quite often observed heavy hemolitic crises (see), the severe bleedings (see) caused by deficit of thrombocytes. The complications connected with need of prolonged use of glucocorticoid hormones and cytostatic drugs are frequent.
At F. — AA. the page should pay special attention to prevention of a recurrence of hemolysis and (or) a trombotsito-liz at emergence inf. diseases, in particular virus (in nek-ry cases appoint small doses of glucocorticoid hormones). Patients shall avoid insolation; administration of drugs, the thrombocytes blocking function is excluded (nag*r., drugs salicylic to - you).
Bibliography: Idelson L. I., D and the Central Committee about in with to and y N. A. and E r m and l chen-to about G. V. Hemolitic anemias, M., 1975, bibliogr.; The guide to hematology, under the editorship of A. I. Vorobyov and Yu. I. Loriye, M., 1979; Savchen
to V. G. and Idelson L. I. Use of a method of Dixon and Rosse for quantitative definition of immunoglobulins on a surface of thrombocytes at a Werlhof's disease, Probl. gema-tolite. and modulation, blood, t. 26, No. 7, page 49, 1981; Dixon R., R osse W. and. E b b e of t L. Quantitative determination of antibody in idiopathic thrombocytopenic purpura, New Engl. J. Med., v. 292, p. 230, 1975; Evans R. S. a. o. Primary thrombocytopenic purpura and acquired hemolytic anemia, evidence for a common etiology, Arch, intern. Med., v. 87, p. 48, 1951; Pirofsky B. Autoimmunization and the autoimmune hemolytic anemias, Baltimore, 1969; Progress in hematology, ed. by E. B. Brown a.
Page of V. Moore, v. 6, p. 82, N. Y. — L., 1969.
V. G. Savchenko.