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-old patient who required monthly blood transfusions. A partial splenectomy was performed to decrease the transfusion requirements and at the same time preserve splenic function. After removal of 80% of splenic tissue 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 regeneration possibly caused by erythrophagocytosis and extramedullary hematopoiesis is the presumed reason for this patient's failure to respond to partial splenectomy.