Pure red cell aplasia in systemic lupus erythematosus

Pure red cell aplasia (PRCA) is a rare hematologic disorder characterized by a severe progressive anemia and a marked decrease or absence of red cell precursors on bone marrow examination. PRCA can occur in the course of many other diseases of which thymoma is the most common. Rarely, PRCA has occurred in patients with connective tissue disorders, the most common being systemic lupus erythematosus (SLE), with 13 cases reported to date in the English literature. In this subset of patients, PRCA can be difficult to manage, and a high rate of mortality has been reported. Multiple therapeutic modalities have been reported, with no clear consensus on the optimal treatment. To date, splenectomy has not been reported in this subset of patients.We describe a patient with SLE who developed PRCA. Prednisone was the initial treatment, but unacceptably high doses were required to control the disease. Intravenous gamma globulin was given with prompt but temporary improvement. The patient subsequently underwent splenectomy with resolution of the PRCA. The patient continues to have a normal hemoglobin 3 years after splenectomy despite no interim therapy. This case represents the 14th case of PRCA occurring in patients with SLE and is the first case of PRCA in SLE treated with splenectomy. Splenectomy should be considered as a therapeutic modality in the management of corticosteroid-refractory PRCA occurring in patients with SLE.