Introduction: Acquired pure red cell aplasia is a rare disorder, usually appearing secondary to various pathologic conditions such as thymoma, systemic autoimmune diseases or in the course of lymphomas. Conventional treatment consists of immunosuppression with corticosteroids, antithymocyte globulin or cyclosporin-A.
Case presentation: 8 weekly courses of rituximab were administered to a patient who presented with pure red cell aplasia secondary to newly diagnosed splenic marginal zone lymphoma. Transfusion independence was achieved after the 6(th) course, and pure red cell aplasia receded completely with therapy.
Conclusion: Pure red cell aplasia may ensue early in the course of splenic marginal zone lymphoma and other low grade lymphomas. Rituximab is a safe and effective alternative treatment for pure red cell aplasia secondary to lymphoproliferative disorders.