Bronchiolitis obliterans after allogeneic hematopoietic stem cell transplantation.
Bronchiolitis obliterans after allogeneic hematopoietic stem cell transplantation.
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DOI：
10.1001/jama.2009.1018
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发表时间：
2009-07-15
期刊：
影响因子：
--
通讯作者：
Pavletic SZ
中科院分区：
文献类型：
--
作者：
Williams KM;Chien JW;Gladwin MT;Pavletic SZ
随着支持治疗的改善，异基因造血干细胞移植（HSCT）后的长期生存率和该手术的适应症都有所增加。因此，由于HSCT而长期存在毒性作用的患者数量有所增加。一种曾经罕见的供体免疫细胞攻击健康宿主组织的情况，称为慢性移植物抗宿主病，已成为一种更常见的现象。当慢性移植物抗宿主病影响肺组织时，闭塞性细支气管炎综合征就会出现。最近的数据表明，闭塞性细支气管炎综合征可能影响高达6%的HSCT接受者，并显着改变生存率，5年总生存率仅为13%。自20多年前首次出现这种疾病以来，这些统计数字没有改善。闭塞性细支气管炎综合征的医学管理进展面临的挑战包括诊断困难和延误以及缺乏指导新疗法的发病机制数据。本文对闭塞性细支气管炎综合征目前的诊断标准进行了批判性评价，并对流行病学、发病机制和可用的治疗方法进行了综述。生存率的提高可能需要早期疾病识别，允许在发生不可逆气道闭塞之前进行疾病的治疗调节。
With improvements in supportive care, both long-term survival following allogeneic hematopoietic stem cell transplantations (HSCTs) and the indications for this procedure have increased. As a result, the number of patients living with long-term toxic effects due to HSCT has increased. A once rare condition of the donor immune cells attacking healthy host tissues, termed chronic graftvs-host disease, has become a more common phenomenon. When chronic graftvs-host disease affects the lung tissue, bronchiolitis obliterans syndrome ensues. Recent data suggest that bronchiolitis obliterans syndrome may affect up to 6% of HSCT recipients and dramatically alters survival, with overall survival of only 13% at 5 years. These statistics have not improved since the first presentation of this disease over 20 years ago. Challenges to the progress of medical management of bronchiolitis obliterans syndrome include difficulties and delays in diagnosis and a paucity of data on pathogenesis to direct new therapies. This article critically evaluates the current diagnostic criteria for bronchiolitis obliterans syndrome and reviews the epidemiology, pathogenesis, and available treatments. Improvements in survival will likely require early disease recognition, allowing for therapeutic modulation of disease prior to the development of irreversible airway obliteration.
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影响因子：
3.3
作者：
Choi, YW;Rossi, SE;McAdams, HP
通讯作者：
McAdams, HP
影响因子：
39.2
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SULLIVAN, KM
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39.2
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24.3
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影响因子：
15.9
作者：
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