Okamura Hiroshi, Nakane Takahiko, Fujino Keizo, Koh Shiro, Yoshimura Takuro, Nishimoto Mitsutaka, Hayashi Yoshiki, Koh Hideo, Nakao Yoshitaka, Nakamae Hirohisa, Hino Masayuki
Hematology, Graduate School of Medicine, Osaka City University.
Rinsho Ketsueki. 2015 Apr;56(4):412-7. doi: 10.11406/rinketsu.56.412.
Myelodysplastic syndrome (MDS) is known to often be complicated by a range of autoimmune diseases. We herein present a case with MDS complicated by cold autoimmune hemolytic anemia (cold AIHA). The patient was a 51-year-old woman. She was diagnosed with MDS (refractory cytopenia with multilineage dysplasia) in May 2009. In January 2010, she underwent unrelated allogeneic bone marrow transplantation but was re-admitted in October 2010 for treatment of relapsed MDS. Despite daily transfusions of red blood cells, her anemia failed to improve. Her laboratory examinations showed a low haptoglobin level and elevation of indirect bilirubin and LDH. The direct Coombs test was positive at a low and at room temperature and cold agglutinin was negative. After confirming the diagnosis of cold AIHA, all transfusion fluids were warmed but her anemia still failed to improve. In addition to the warmed transfusion fluids, we administered corticosteroids, immunosuppressive agents and high-dose intravenous immunoglobulin infusions. This management strategy ameliorated the patient's hemolytic anemia. To our knowledge, MDS cases complicated by cold AIHA are rare. Our patient thus provides a valuable contribution to medical knowledge.
已知骨髓增生异常综合征（MDS）常并发一系列自身免疫性疾病。我们在此报告一例MDS合并冷凝集素介导的自身免疫性溶血性贫血（冷凝集素介导的冷AIHA）的病例。患者为一名51岁女性。她于2009年5月被诊断为MDS（伴有多系发育异常的难治性血细胞减少症）。2010年1月，她接受了非亲缘异基因骨髓移植，但于2010年10月因复发性MDS再次入院治疗。尽管每天输注红细胞，但其贫血仍未改善。她的实验室检查显示触珠蛋白水平低，间接胆红素和乳酸脱氢酶升高。直接抗人球蛋白试验在低温和室温下呈阳性，冷凝集素为阴性。在确诊冷凝集素介导的冷AIHA后，所有输血液体均进行了加温处理，但她的贫血仍未改善。除了加温输血液体外，我们还给予了皮质类固醇、免疫抑制剂和大剂量静脉注射免疫球蛋白。这种治疗策略改善了患者的溶血性贫血。据我们所知，MDS合并冷凝集素介导的冷AIHA的病例很少见。因此，我们的患者为医学知识做出了宝贵贡献。
