Successful Treatment of a Patient With Pure Red Cell Aplasia With Prednisone, Cyclosporine and Antithymocyte Globulin.

Introduction

Pure red cell aplasia is a rare disease characterized by severe anemia with reticulopenia, along with selective aplasia of the erythroid cell line in the marrow. The acquired form can be transient and self-limited or sustained and chronic. [1] Transfusion of packed red cells is the usual initial therapy. Thymectomy is usually performed when there is thymic enlargement in order to obtain a diagnosis and possibly reactivate hematopoiesis in the bone marrow. [2] Multiple immunomodulating treatments also have been administered in this disorder. Glucocorticoids have been used with varying efficacy and substantial side effects in large, prolonged dosing schedules. Immunosuppressive therapies such as cyclosporine (CSA) have been used with limited success. Antithymocyte globulin (ATG) as a single agent has been tried with mixed results. [3] Interestingly, these treatments have generally been used as single agents or as second- or third-line therapy. In our review of the literature, we did not encounter any report of the use of a combination of ATG, CSA and steroids, which is frequently employed for management of aplastic anemia. In this case, we report a sustained remission of acquired pure red cell aplasia treated with this combination therapy.

Case Report

The patient was a 20-year-old white woman diagnosed with pure red cell aplasia 1 year prior to appearing at our institution. She presented with fatigue and …