A Case of Pure Red Cell Anemia Complicated by Antiepileptic Drugs

A 12-year-old boy developed pure red cell anemia(PRCA) during a combination therapy of antiepileptic drugs(AEDs) for epilepsy. His complex partial seizure was intractable to monotherapy. During 7 months of treatment, he was treated with Vigabatrin, Carbamazepine and Valproate. While switching from Carbamazepine to Valproate, he presented anemia but with no jaundice. His hemoglobin was 4.1g/dl bone-marrow biopsy revealed erythroid hypoplasia with normal myelopoiesis and megakaryocytopoiesis, indicating PRCA. Rapid recovery from PRCA was observed 1 month after discontinuation of Valproate, without immunosuppressive therapy. Although the hematologic toxicity of AEDs is well documented, isolated cessation of red cell production is uncommon. Our observation suggests that the synergistic toxicity caused by Valproate and Carbamazepine may induce PRCA through the inhibitory effect beyond the differentiation stage of BFU-E and CFU-E.

A Case of Diabetes Mellitus due to Hemochromatosis in Patient with CPRCA

Iron overload from repeated blood transfusions in patients with hematologic problems is a problem of clinical significance, because eventually it can lead to secondary hemochromatosis with diabetes. So it is important to diagnose iron overload early and to restrict blood transfusions in these patients. Recently, we experienced one case of secondary hemochromatosis with diabetes in patient wi th congenital pure red cell anemia, diagnosed by serum ferritin level, TIBC, transferrin saturation, random blood sugar, C-peptide and insulin level. This patient had received 30,000ml of RBCs for 9 years. Treatments with insulin mixed spitting therapy because of insulin dependent diabetes clinically, restriction of blood transfusion and steroid therapy for maintenance of Hemoglobin level were done just after discharge. We report this one case with a brief review of the literature.