FAILURE OF PARTIAL SPLENECTOMY TO AMELIORATE THE ANEMIA OF PYRUVATE-KINASE DEFICIENCY

The authors report a case of pyruvate kinase deficiency in a 4-year-ol d patient who required monthly blood transfusions. A partial splenecto my was performed to decrease the transfusion requirements and at the s ame time preserve splenic function, After removal of 80% of splenic ti ssue there was no amelioration of her transfusion needs. She responded well to subsequent total splenectomy performed 6 months later, and is currently well and transfusion independent. Rapid splenic regeneratio n possibly caused by erythrophagocytosis and extramedullary hematopoie sis is the presumed reason for this patient's failure to respond to pa rtial splenectomy. Copyright (C) 1997 by W.B. Saunders Company.