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获得性再生障碍性贫血的管理

Management of acquired aplastic anaemia.

作者信息

Marsh Judith C W

机构信息

Department of Haematology, St George's Hospital Medical School, Cranmer Terrace, London SW17 ORE, UK.

出版信息

Blood Rev. 2005 May;19(3):143-51. doi: 10.1016/j.blre.2004.06.002.

Abstract

Outcome of patients with aplastic anaemia (AA), whether treated with allogeneic BMT or immunosuppressive therapy has steadily increased over the last three decades. However, there is a difference in quality of outcome between these two therapeutic modalities. There is no plateau for survival after ATG as patients are at later risk of transformation to myelodysplasia (MDS) or acute myeloid leukaemia (AML), paroxysmal nocturnal haemoglobinuria and relapse of their aplasia. In contrast, AA patients are not at risk of these later complications if they have undergone successful bone marrow transplantation. Long term survival after HLA identical sibling BMT is 80-90%, but GVHD and graft rejection remain to be addressed. The results of unrelated donor BMT for AA have shown considerable improvement over the last five years. Difficulties remain for those patients who fail immunosuppressive therapy and in whom BMT is not possible, since alternative immunosuppressive agents have so far proven to be somewhat disappointing.

摘要

在过去三十年中,再生障碍性贫血(AA)患者接受异基因骨髓移植(BMT)或免疫抑制治疗后的预后稳步改善。然而,这两种治疗方式的预后质量存在差异。接受抗胸腺细胞球蛋白(ATG)治疗后患者的生存率没有达到平台期,因为患者后期有转化为骨髓增生异常综合征(MDS)或急性髓系白血病(AML)、阵发性睡眠性血红蛋白尿症以及再生障碍性贫血复发的风险。相比之下,接受成功骨髓移植的AA患者不存在这些后期并发症的风险。人类白细胞抗原(HLA)相合同胞骨髓移植后的长期生存率为80%至90%,但移植物抗宿主病(GVHD)和移植物排斥问题仍有待解决。过去五年中,非血缘供者骨髓移植治疗AA的结果有了显著改善。对于那些免疫抑制治疗失败且无法进行骨髓移植的患者来说,困难依然存在,因为迄今为止,替代免疫抑制剂的效果有些令人失望。

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