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European Society for Paediatric Haematology and Immunoloy Abstracts

AUTOIMMUNE HEMOLYTIC ANEMIA-THREE CASES REPORT. 92

Objective: Immune hemolytic anemia constitutes a diverse spectrum of disease entities that triggers the production, the deposition of specific antibody and fixation of complement on the target cell erythrocyte. The result is intravascular destruction of the red cell. Two clinical entities exist - one with cold reacting and the other one with warm reacting antibodies.

Material and methods: We present three patients with AIHA.Case 1. 13-year old girl manifested mild AIHA with warm erythrocyte non specific IgG antibodies. Monospecific direct Coombs test anti IgG was positive. Indirect Coombs test at 4 °C and 37 °C was positive. Antinuclear antibodies, cryoglobulines and circulated immune complexes were found positive. ANCA MPO were increased. The presence of LE cells was not proven. The disease had a prolonged course and deterioration. Case 2. 8-year old boy who manifested mononucleosis syndrome and severe AIHA. Direct Coombs test negative, Donath-Landsteiner test negative. Indirect Coombs test positive at 4 °C and negative at 37 °C. IgM EBV-VCA 60 Au/ml, IgG EBV-VCA 190 Au/ml, IgM and IgG anti EBV-EBNA negative. Circulating immune complexes, ANCA PRO 3, cryoglobulins increased. Case 3. 10-month old child manifested severe AIHA, 3 days after immunization (diphtheria, poliomyelitis, pertussis and tetanus). Direct Coombs test positive, indirect Coombs tests lightly positive at 37 °C. On patient's erythrocytes components of complement and anti-i agglutinins were confirmed active at room temperature and at 37 °C.

Results: All the patients were treated with corticosteroids, two of them shortly and successfully, while the girl is still on therapy (8 months) with a second attack of severe persistent AIHA.

Conclusion: Different triggering factors of AIHA in childhood are confirmed in our report such as EBV infection, immunization and immune connective tissue disease.

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Culic, S., Kuljis, D., Martinic, R. et al. AUTOIMMUNE HEMOLYTIC ANEMIA-THREE CASES REPORT. 92. Pediatr Res 41, 761 (1997). https://doi.org/10.1203/00006450-199705000-00111

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