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CASES WITH AUTOIMMUNE HEMOLYTIC ANEMIAS (AIHA) IN CHILDREN 86

The data from 24 cases of children with AIHA diagnosed and treated in Children's Oncohematology Clinic in Sofia, Bulgaria, between 1979-1996 are retrospectively analyzed. Four patients were classified as secondary(symptomatic) AIHA (LED-1 case, Wiskott-Aldrich-1, AIH-2). The rest were patients with idiopathic forms. Among the latter 16 of the patients had an acute course of the illness and 4 had a chronic disease. Most of the children were boys- 17 (85%). There was a prevalence of children under the age of 4 (16 patients - 80%). Seven of all patients were infants (35%) and 5 of them were below 6 months old (71.4%). Five patients had Evans syndrome. DAT was positive in 19 of the cases at the time of diagnosis and in only one the rest became positive on day 18th. Warm antierythrocyte antibodies and parallel complement C3- and C4- fixation was found. In 2 of the cases with marked intravascular hemolysis there was evidence of pure complemental destruction of erythrocytes. Seventy percent of the patients with idiopathic AIHA were cured, and these were children with acute form of the disease and good response to corticosteroids. For the whole period (1979-1996) six patients died of idiopathic AIHA and they were mostly infants, two of them during the last 6 years (1990-1996). The reevaluated data showed that there is a dramatic improvement of the therapeutic response in patients with AIHA due to better treatment and support.