Acquired Adult Pure Red Cell Aplasia

Pure red cell aplasia (PRCA) is a rare hematological disorder characterized by severe anemia due to a selective failure of erythropoiesis. PRCA exhibits diverse epidemiological patterns worldwide. PRCA is classified into congenital, primary, and secondary forms. Congenital PRCA, such as Diamond-Blackfan anemia, involves genetic abnormalities, whereas transient erythroblastopenia of childhood lacks a clear etiology. Acquired PRCA in adults can result from various factors, including drugs, infections, autoimmune diseases, malignancies, and pregnancy. The pathophysiology of PRCA varies; parvovirus B19 directly damages erythroid precursors, while other cases involve immunologically mediated mechanisms, including antibody-mediated and T-cell-mediated processes. Clinical manifestations often appear in advanced stages, with symptoms of anemia. Laboratory findings show severe reticulocytopenia, normocytic normochromic anemia, and diminished erythroblasts in the bone marrow. Treatment strategies involve addressing the underlying cause, discontinuing offending medications, and thymectomy for thymoma cases. Immunosuppressive therapy with cyclosporine is the first-line treatment. Other therapies may be considered for refractory cases, including cyclophosphamide, azathioprine, IV immunoglobulin, plasmapheresis, rituximab, and alemtuzumab. Spontaneous remission occurs in a minority of patients, while most respond to immunosuppressive therapy. The prognosis varies based on the type of PRCA and underlying conditions.