PB1970 AUOTOIMMUNE HEMOLITIC ANEMIA (AIHA) IN CHILDHOOD. IS PRIMARY IMMUNE DISORDERS MORE OFTEN THAN EXPECTED IN ETIOLOGY OF SECONDARY AIHA

Abstract

Background:Auotoimmune hemolitic anemia (AIHA) results from redcell destruction due to circulating antibodies against redcell membrane antigens. They are classified etiologically into primary and secondary such as primary immune disorders (PID), infectious disease and connective tissue disorders (CTD).Aims:Herein, we aimed to evaluation of etiology in cases with AIHA.Methods:62 cases diagnosed on positive direct antiglobulin test (DAT)at department of pediatric hematology in Erciyes University Hospital were included in the study and their records have been retrosectively examined.Results:Mean age of all cases was 61.9 ± 51.9 months (range of 1‐92 months). The cases showed a male predilection (M:F = 1.2). The results showed that 34 of 62 cases had primary 28 secondary AIHA (12 PID, 7 infections, 4 CTD, 3 Hodgkin disease, 2 endocrinopathies) respectively. Mean Hb value in cases with AIHA was 6.9 ± 2.5 g/dl and mean Hb value in cases with secondary AIHA was 6.8‐2.6 g/dl. All of them had mean low level of haptoglobulin (7.2 mg/dl in primary AIHA, 6.2 mg/dl in secondary AIHA) and they had mean high level of total bilurubin (4.9 mg/dl in primary AIHA and 1.4 mg/dl in secondary AIHA). There was no significant difference of mean Hb value in two groups (p = 0.783) but there was significant difference of mean bilurubin value in two groups (p = 0.001). One Case with secondary AIHA had IgM cold autoantibody and 61 cases had IgG warm autoantibody. The cases with primary AIHA had more strong positive IgG autoantibody than the cases with secondary AIHA and there was significant difference of IgG autoantibody positivity in two groups (p = 0.044).Summary/Conclusion:AIHA presented with severe anemia and laboratory evidence of marked hemolysis. Especially, PID is more often than expected in etiology of secondary AIHA.