Atallah Jane, Al Alousi Yarub, Guevara Nehemias, Statnii Iurii, Nassar Sameh, Vigoda Ivette
Department of Medicine, St. Barnabas Hospital Health System, Bronx, NY, USA.
Department of Hematology/Oncology, St. Barnabas Hospital Health System, Bronx, NY, USA.
Case Rep Oncol. 2024 Jan 9;17(1):69-74. doi: 10.1159/000535636. eCollection 2024 Jan-Dec.
Pure red cell aplasia (PRCA) is a rare bone marrow failure characterized by normocytic anemia and severe reticulocytopenia.
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.
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.
纯红细胞再生障碍性贫血（PRCA）是一种罕见的骨髓衰竭，其特征为正细胞性贫血和严重的网织红细胞减少。
我们描述了一名38岁女性严重贫血的病例。进一步检查发现贫血病因是PRCA。随后她在骨髓活检中被诊断为B细胞型慢性淋巴细胞白血病（CLL）。患者拒绝输血支持。尽管接受了利妥昔单抗和类固醇治疗，但病情未改善并最终死亡。
我们的病例是PRCA的一种不寻常表现，导致了CLL的诊断。PRCA是CLL中极其罕见的贫血原因，约1%的患者会出现。其发病机制被认为是免疫介导的。CLL中PRCA的治疗包括使用类固醇、环孢素和利妥昔单抗进行免疫抑制治疗，但在大多数情况下通常难以治愈。
