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肺移植接受者患有端粒介导的肺纤维化,患有血液学并发症的风险增加

Stefanie J Hannan¹, Carlo J Iasella², Rachel M Sutton¹

¹Division of Pulmonary, Allergy and Critical Care Medicine, School of Medicine University of Pittsburgh, Pittsburgh, Pennsylvania, USA; Lung Transplant Research Center, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.

American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons

|July 1, 2023

概括

具有短端粒长度 (TL) 的异常性肺纤维化肺移植接受者面临更高的骨髓功能障碍和血液学并发症的风险. 移植前的TL和遗传检测可以识别有风险的患者,以便更好地管理.

科学领域:

肺部医学肺部医学
血液学血液学血液学
遗传学遗传学是一个

背景情况:

异常性肺纤维化肺移植接受者 (IPF-LTRs) 经常表现出短端粒长度 (TL) 和罕见的端粒基因变异.
非移植患者的短TL与骨髓 (BM) 功能障碍有关.

研究的目的:

调查假设,具有短TL和/或罕见变异的IPF-LTRs具有增加移植后血液学并发症风险的假设.
为了识别IPF-LTRs易受肺移植后不良血液学结果.

主要方法:

对72名IPF-LTR和72名年龄匹配的非IPF-LTR对照进行了回顾性队列研究.
通过流细胞计和光现场杂交 (FlowFISH) 来测量TL.
使用全基因组测序或向面板进行遗传评估;用于TL分析的TelSeq软件.

主要成果:

大多数IPF-LTR具有短TL;26%携带罕见变体.
短TL的IPF-LTR显示,由于细胞衰竭 (P = .0375) 和需要活检的BM功能障碍 (29%对4%,P = .0003) 导致免疫抑制中止的比率更高.
短TL和罕见变体的IPF-LTR需要更多的输血和生长因子支持;短TL,罕见变体和低移植前血小板预测了BM功能障碍.

结论:

在移植前的TL测量和对端粒基因变异的基因测试可以识别高风险的IPF-LTRs.
这些发现支持分层分层的肺移植候选人,他们患有端粒介导的肺纤维化.

关键词:

BM 失败 BM 失败 BM 失败肺部移植肺部移植肺部纤维化肺部纤维化端粒是什么意思端粒是什么意思

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