Pure red cell aplasia: review of treatment and proposal for a treatment strategy

The management of pure red cell aplasia (PRCA) continues to challenge clinical investigators because the pathophysiology is heterogeneous and poorly understood. There are five treatment regimens that have established efficacy for patients with chronic PRCA. In patients with congenital hypoplastic anemia the best results have been reported using corticosteroids. Cyclosporine A is recommended as the treatment of choice in acquired PRCA. High-dose intravenous immunoglobulin therapy is highly effective in PRCA associated with parvovirus B19 infections and impaired IgG-antibody response. Treatment failures may be successfully managed with horse anti-human thymocyte globulin or cyclophosphamide plus corticosteroids. The potential of hematopoietic growth factors in the treatment of PRCA awaits further studies.