Successful Treatment of Pure Red Cell Aplasia (PRCA) Associated to Phenitoin without Blood Transfusions in a Jehovah’s Witness Patient.

A 17 year-old Jehovah’s Witness girl was admitted to the neurosurgery ward with a subarachnoid hemorrhage Fisher IV, Hunt and Hess II, due to an arteriography-confirmed aneurism of the left posterior comunicating artery (LPCA). CT scan showed hemorrhages of the left lateral, 3^rd and 4th ventriculi. Her blood counts were normal. She underwent surgery and during the procedure she developed neurologic deterioration due to brain edema. The procedure was stopped and she was started on prophylactic Phenitoin. Her hemoglobin levels began to drop progressively until they reached 3.6 gr/dL with 0.8% reticulocytes while her platelet and WBC counts remained within normal ranges (278 and 5.9 × 10⁹/L respectively) with a normal differential count. No bleeding site was found, and hemorrhage was discarded as cause of the anemia. The serum iron was 218, the IBC 213 and the saturation index 102.5 (37.3%), all within normal values. The bone marrow aspirate showed a slight increase of the cellularity with normal megakariocytes and granulocytes with slight eosinophilia and a total absence of the erythroid series. The lymphocytes were also normal. This image was consistent with a PRCA. The existence of a thymoma as well as several viral infections including Parvovirus, EBV, HIV, HB and HC were discarded as cause of the PRCA. Phenitoin was stopped and treatment with steroids, cyclosporine, mesterolone and erythropoietin 8,000 U/d SC was started. Despite the evidence of very deep anemia with dispnea and prostration, which required red cell transfusions, the patient and her parents absolutely refused them for religious reasons and the supportive treatment was enhanced. Her clinical status improved after 48 hours and the Hb began to increase progressively. She was discharged one month later with a Hb of 13.1 gr/dL, Ht 39.6%, with a corrected reticulocyte count of 1.8%, 6.2 × 10⁹/L WBC with a normal differential count and 284 × 10⁹/L BP. Even though no tests could be done to confirm phenitoin as the triggering factor of the PRCA, the temporal association between its use and the development of the anemia very strongly suggests a causal association even though it can not be assured. The results we had with erythropoietin, high-dose iron, oxygen and parenteral feeding were very rewarding. Jehovah’s Witnesses patients with severe anemia like this one have a poor prognosis and pose a strong therapeutic challenge that may discourage the medical staff to continue a struggle that seems hopeless. We feel that by accepting it and sharing experiences like this one we can help these patients who will rather die than accept blood derivatives.