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双侧肺移植治疗特发性支气管中心性肉芽肿病终末期闭塞性细支气管炎。

Bilateral lung transplantation for Castleman disease with end-stage bronchiolitis obliterans.

机构信息

Wuxi Lung Transplant Center, Department of Thoracic Surgery, Wuxi People's Hospital affiliated to Nanjing Medical University, Wuxi, China.

Department of Lung Transplantation, Center of Lung Transplantation, Center of Respiratory Diseases, China-Japan Friendship Hospital, Beijing, China.

出版信息

Clin Transplant. 2022 Jan;36(1):e14496. doi: 10.1111/ctr.14496. Epub 2021 Oct 21.

Abstract

Bronchiolitis obliterans (BO) is a severe complication of Castleman disease (CD), a rare lymphoproliferative disease with unclear pathogenesis. Currently, there are no reports on the safety or outcomes of bilateral lung transplantation in patients with BO due to CD. This study aimed to characterize the clinical manifestations and features of BO and CD. We retrospectively analyzed the medical records of six consecutive patients with BO and CD who underwent bilateral lung transplantation between December 2012 and December 2020. The average age of patients at lung transplantation was 33 ± 15 years, and the age range of patients at diagnosis of CD was about 9-56 years. The body mass index was 15.2 ± 1.9 kg/m . The average time from diagnosis to lung transplantation was 4.1 ± 2.7 years. All the patients had unicentric CD (UCD); five had concomitant paraneoplastic pemphigus, and four received extracorporeal membrane oxygenation during surgery. The average hospital stay was 51 ± 53 days. Infection was the most common postoperative complication. CD did not recur in any of the patients. Thus, bilateral lung transplantation is a viable and safe treatment for selected patients with CD and BO, which can improve the quality of life and prolong survival.

摘要

闭塞性细支气管炎(BO)是卡斯特曼病(CD)的一种严重并发症,CD 是一种罕见的淋巴增生性疾病,其发病机制尚不清楚。目前,尚无关于 CD 导致 BO 患者行双侧肺移植的安全性或结局的报道。本研究旨在描述 BO 和 CD 的临床表现和特征。我们回顾性分析了 2012 年 12 月至 2020 年 12 月期间连续 6 例接受双侧肺移植的 BO 和 CD 患者的病历。肺移植时患者的平均年龄为 33 ± 15 岁,CD 诊断时患者的年龄范围约为 9-56 岁。体重指数为 15.2 ± 1.9 kg/m²。从诊断到肺移植的平均时间为 4.1 ± 2.7 年。所有患者均为单中心 CD(UCD);5 例合并副肿瘤性天疱疮,4 例在手术中接受体外膜氧合。平均住院时间为 51 ± 53 天。感染是最常见的术后并发症。所有患者均未复发 CD。因此,双侧肺移植是治疗 CD 和 BO 患者的一种可行且安全的方法,可以提高生活质量并延长生存时间。

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