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再生障碍性贫血的病理生理学和治疗的当前概念。

Current concepts in the pathophysiology and treatment of aplastic anemia.

作者信息

Young Neal S

机构信息

1Hematology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD.

出版信息

Hematology Am Soc Hematol Educ Program. 2013;2013(1):76-81. doi: 10.1182/asheducation-2013.1.76.

Abstract

Historically viewed in isolation as an odd, rare, and invariably fatal blood disease, aplastic anemia is now of substantial interest for its immune pathophysiology, its relationship to constitutional BM failure syndromes and leukemia, and the success of both stem cell transplantation and immunosuppressive therapies in dramatically improving survival of patients. Once relegated to a few presentations in the red cell and anemia sessions of the ASH, the Society now sponsors multiple simultaneous sessions and plenary and scientific committee presentations on these topics. This update emphasizes developments in our understanding of immune mechanisms and hematopoietic stem cell biology and new clinical approaches to stem cell stimulation as a therapy, alone and in combination with conventional suppression of the aberrant immune system.

摘要

再生障碍性贫血在历史上一直被孤立地视为一种奇特、罕见且必然致命的血液疾病,如今,因其免疫病理生理学、与先天性骨髓衰竭综合征及白血病的关系,以及干细胞移植和免疫抑制疗法在显著提高患者生存率方面的成功,它备受关注。再生障碍性贫血曾仅在ASH的红细胞和贫血会议上有少量展示,如今该协会针对这些主题同时举办了多个会议以及全体会议和科学委员会报告。本更新内容着重介绍了我们在免疫机制和造血干细胞生物学理解方面的进展,以及作为一种疗法单独或与传统的异常免疫系统抑制联合使用的干细胞刺激新临床方法。

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