Introduction: Pure red cell aplasia (PRCA) is a rare bone marrow failure characterized by normocytic anemia and severe reticulocytopenia. Case Presentation: We describe the case of a 38-year-old female who presented with severe anemia. Further investigation revealed the etiology of anemia to be PRCA. She was subsequently diagnosed with chronic lymphocytic leukemia (CLL) B-cell type on bone marrow biopsy. The patient refused blood transfusion support. She failed to improve and expired despite treatment with rituximab and steroids. Conclusion: Our case is an unusual presentation of PRCA that led to the diagnosis of CLL. PRCA is an extremely rare cause of anemia in CLL, occurring in around 1% of patients. The pathogenesis is thought to be immune mediated. Treatment of PRCA in CLL involves immunosuppressive therapy with steroids, cyclosporine, and rituximab, yet it is usually refractory in most cases.
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