Abstract
Idiopathic AIHA in childhood is usually an acute self-limited disease, responding well to steroids and transfusion. In severe refractory cases, immunosuppressive agents may be of help, but the slow onset of effect (usually weeks) limits their usefulness. Presented with a 17 year-old boy with severe acute AIHA unresponsive to prednisone (10 mg/kg/d), exchange transfusion & splenectomy, we performed PE using the IBM model 2997 Blood Cell Separator. Before PE he required 20 u PRBC over 14 days to keep his Hb > 4 g/dl. Post-PE his Hb remained stable for 5 days. Azathioprine was begun, but when his Hb fell 2 days later a 2nd PE was performed. Again there was abrupt stabilization of Hb levels followed by return to normal over the next 3 weeks, without further transfusion. Total volumes of blood processed in the 2 PE were 8500 & 8250 ml, representing 4240 & 4140 ml plasma (incl. extracellular fluid). Transient coagulation defects were observed, but were corrected by fresh frozen plasma infused post-PE; no bleeding problems developed. Our observations in this case suggest that antibody removal by PE enabled prompt control of severe hemolysis, ‘buying time’ until the immunosuppressive effect of azathioprine took place. Further trial of this combined therapy seems warranted in cases of acute disease refractory to large doses of steroids, and perhaps in younger children with chronic drug-dependent disease prior to consideration of splenectomy.
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Naiman, J., Bernstein, M. & Schneider, B. 841 INTENSIVE PLASMA EXCHANGE (PE) IN REFRACTORY ACUTE AUTOIMMUNE HEMOLYTIC ANEMIA (AIHA). Pediatr Res 15 (Suppl 4), 582 (1981). https://doi.org/10.1203/00006450-198104001-00866
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DOI: https://doi.org/10.1203/00006450-198104001-00866