Yasuyama Masako, Kawauchi Kiyotaka, Takei Kazuhiro, Ogasawara Toshie, Ohkawa Shinichiro
Department of Medicine, Tokyo Women's Medical University Daini Hospital.
Rinsho Ketsueki. 2004 Jan;45(1):66-71.
The patient was a 47-year-old man who was diagnosed in 1989 as having chronic myelogenous leukemia (CML). He had been treated with interferon-alpha (IFN-alpha) and hydroxyurea. In August 1999, he was admitted to our hospital for examination of severe anemia and increased platelet count. On admission, his hemoglobin level was 6.3 g/dl, reticulocyte count was 0.7%, WBC count was 5,100/microliter, and platelet count was 57.3 x 10(4)/microliter. Bone marrow aspiration showed myeloid hyperplasia and near absence of erythroblasts. Bone marrow karyotype analysis showed a Ph chromosome with additional abnormalities. Pure red cell aplasia (PRCA) with accelerated-phase CML was considered. The IFN-alpha therapy was discontinued. Hydroxyurea at an increased dosage was effective in controlling the CML. In contrast, administration of cyclosporin A was not effective for the PRCA. The patient's condition was later complicated by acute hepatitis C virus infection. The IFN-alpha was restarted to control the CML and hepatitis. The patient remained erythroblastopenic and transfusion-dependent for more than 2 years. Association of CML and PRCA is rare. We discuss the mechanisms underlying PRCA occurring during the course of CML.
该患者为一名47岁男性，1989年被诊断为慢性粒细胞白血病（CML）。他曾接受过α干扰素（IFN-α）和羟基脲治疗。1999年8月，他因严重贫血和血小板计数增加入院检查。入院时，他的血红蛋白水平为6.3 g/dl，网织红细胞计数为0.7%，白细胞计数为5100/微升，血小板计数为57.3×10⁴/微升。骨髓穿刺显示骨髓增生，几乎没有成红细胞。骨髓核型分析显示有费城染色体及其他异常。考虑为慢性粒细胞白血病加速期合并纯红细胞再生障碍性贫血（PRCA）。停用α干扰素治疗。增加剂量的羟基脲对控制慢性粒细胞白血病有效。相比之下，环孢素A对PRCA无效。该患者后来并发急性丙型肝炎病毒感染。重新开始使用α干扰素以控制慢性粒细胞白血病和肝炎。该患者持续成红细胞缺乏且依赖输血超过2年。慢性粒细胞白血病与PRCA的关联罕见。我们讨论了慢性粒细胞白血病病程中发生PRCA的潜在机制。
