再生障碍性贫血的临床管理。
Clinical management of aplastic anemia.
机构信息
Division of Medical Oncology, Department of Medicine, The Johns Hopkins School of Medicine, 720 Rutland Avenue, Baltimore, MD 21205, USA.
出版信息
Expert Rev Hematol. 2011 Apr;4(2):221-30. doi: 10.1586/ehm.11.11.
Abstract
Acquired aplastic anemia is a potentially fatal bone marrow failure disorder that is characterized by pancytopenia and a hypocellular bone marrow. Hematopoietic stem-cell transplantation or bone marrow transplantation (BMT) is the treatment of choice for young patients who have a matched sibling donor. Immunosuppression with either anti-thymocyte globulin and cyclosporine or high-dose cyclophosphamide is an effective therapy for patients who are not suitable BMT candidates owing to age or lack of a suitable donor. Results of BMT from unrelated and mismatched donors are improving, but presently this treatment option is best reserved for those patients who do not respond, relapse or develop secondary clonal disorders following immunosuppressive therapy. Efforts are currently underway to both improve immunosuppressive regimens and to expand the application of BMT.
摘要
获得性再生障碍性贫血是一种潜在致命的骨髓衰竭疾病，其特征是全血细胞减少和骨髓细胞减少。造血干细胞移植或骨髓移植（BMT）是有匹配的同胞供体的年轻患者的首选治疗方法。对于由于年龄或缺乏合适供体而不适合 BMT 候选者的患者，用抗胸腺细胞球蛋白和环孢菌素或大剂量环磷酰胺进行免疫抑制是一种有效的治疗方法。无关和不匹配供体的 BMT 结果正在改善，但目前这种治疗选择最好保留给那些在免疫抑制治疗后无反应、复发或发生继发性克隆疾病的患者。目前正在努力改善免疫抑制方案并扩大 BMT 的应用。
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