SEKUNDÁRNÍ HEMOFAGOCYTUJÍCÍ SYNDROMPŘI SYSTÉMOVÉM ONEMOCNĚNÍ

Virus-Associated Hemophagocytic Syndrome in Systemic Disease

20 year old man 2 years treated for the seropositive rheumatoid arthritis was admitted for fever accompanied withjaundice, anemia and leukopenia. The underlying disease has been compensated already for long period of time,before his admission only Prednison (in the dose of 5 mg daily) and Methotrexat (15 mg once a week) was given.His physical examination of admission was without any significant abnormalities, out of the routine laboratoryexamination the value of leukocytes count was 2.1 x 109/L, erythrocytes 3.7 x 1012/L, hemoglobin 95 g/l, hematocrit0.29, platelets 156 x 109/L. Since admission to hospital the hepatic enzymes ALT, AST, GMT, ALP were about tentimes elevated comparing to normal values, the coagulation examination has shown the decrease of Quick test to55 %. With respect to the permanent leukopenia the bone marrow aspiration was taken with the finding of the increasenumber the RES elements (18.4%) with the signs of hemophagocytosis. The phagocytic reticulum absorbs bloodelements erythrocytes, normoblasts, granulocytes, platelets. According to the literature experience we started thecombination of the immunosupresive treatment consisting of corticosteroids and Cyclosporine. Already the dayfollowing the application of the high dose of corticosteroids the fever subsided, icterus went away gradually withthe normalization of the liver tests. After 20 days of hospitalisation the patient was discharged in good shape. Now,after 4 months the is stabilized on the follow-up treatment of Prednison a Cyklosporine.

Key words:
systemic autoimmune disease, hemophagocytic syndrome, rheumatoid arthritis, anemia, leukopenia,