Abstract
The identification of patients with thalassemia major who will clearly benefit from splenectomy is often difficult. Modell (Br Med Bull 32:270,1976) has evaluated transfusion requirements based upon weight, mean hemoglobin level and amount of administered whole blood. We have modified this index using the pre-transfusion Hb level and quantity of packed red cells. Transfusion requirements, expressed as the volume of transfused packed RBC's in one year divided by weight at mid-year, were studied in 48 patients with thalassemia major and intact spleens and 18 patients after splenectomy. The patients with spleens (3-24 years old) required 131-367 ml/kg/yr (mean 223) to maintain a pre-transfusion Hb level of 7.8-9.8 g/dl. The 18 patients (5-28 years old) who underwent splenectomy required 80-155 ml/kg/yr (mean 126) to maintain a pre-transfusion Hb level of 8.3-10.6 g/dl. In 8 patients studied before and at least six months after splenectomy, the amount of administered blood decreased 24-74% (mean 48%), despite an increase in the mean pre-transfusion Hb level from 8.5 to 9.6 g/dl. Post-splenectomy transfusion requirements in all eight patients were in the predicted range. As illustrated by these data, the transfusion index allows easy identification of those patients with Cooley's anemia who will benefit from splenectomy and permits prediction of the decrease in blood requirements following splenectomy.
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Cohen, A., Markenson, A. & Schwartz, E. 590 TRANSFUSION REQUIREMENTS AND SPLENECTOMY IN COOLEY'S ANEMIA. Pediatr Res 12 (Suppl 4), 462 (1978). https://doi.org/10.1203/00006450-197804001-00595
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DOI: https://doi.org/10.1203/00006450-197804001-00595