Abstract
Fourteen patients with AIHA between the ages of 10 weeks to 18 years were investigated. Of these 10 had warm reactive autoantibodies (6 anti-dl, 1 anti-nl, 1 anti-Rho, 1 non-specific, 1 Coombs negative IgGl antibody) and 4 had cold reactive autoantibodies (3 anti-l and 1 non-specific antibody). At the time of initial diagnosis the hemoglobin levels varied from 2.8 to 8.7 gm/dl. Four patients had reticulocytopenia at the onset. In 2 patients the haptoglobin levels were normal. One patient received no treatment, one only required a blood transfusion and 12 were treated with corticosteroids of which 11 responded successfully. The patient who failed to respond to corttcosteroids also did not respond to exchange transfusion and immunosuppresslve drugs but responded following splenectomy. Eleven were idiopathic and 3 had the following associated conditions: mycoplasma pneumonitis, systemic lupus erythematosus and a “panhematoimmunopathy” (anti-neutrophii autoantibody, anti-platelet autoantibody and anti-dl). In most of these patients the course of AIHA was transient, due to warm autoantibodies and responsive to steroids. Coombs negative AIHA is an uncommon entity requiring detailed immunohematologic investigations including the use of the autoanalyzer and elution studies for its identification.
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Shende, A., Lanzkowsky, P. & Hsu, T. CLINICAL ASPECTS OF AUTOIMMUNE HEMOLYTIC ANEMIA IN CHILDREN (AIHA). Pediatr Res 11, 480 (1977). https://doi.org/10.1203/00006450-197704000-00663
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DOI: https://doi.org/10.1203/00006450-197704000-00663