造血干细胞移植后闭塞性细支气管炎综合征行肺移植治疗的临床疗效观察

Clinical observation of lung transplantation in patients with obstructive bronchiolitis syndrome after hematopoietic stem cell transplantation

【摘要】目的探讨肺移植治疗造血干细胞移植术后闭塞性细支气管炎综合征（bronchiolitis obliterans syndrome，BOS）的手术时机和术后并发症。方法收集2012年1月至2015年9月期间，造血干细胞移植后闭塞性细支气管炎综合征患者接受肺移植的临床资料。结果8例造血干细胞移植术后闭塞性细支气管炎综合征患者经过评估，接受肺移植手术治疗。受者年龄为（30．88±12．12）岁（13-47岁），在诊断为BOS后（3．44±1．29）年（1～5年）接受了肺移植，4例患者接受了双肺移植，2例患者接受了右单肺移植，1例患者因漏斗胸，在双肺移植同期行了漏斗胸矫治术，另1例13岁儿童接受了双侧肺叶移植术。肺移植术后2例受者分别于术后第2天和第9天发生了急性排斥反应。4例受者术后早期经历了曲霉菌感染，1例为肺实质侵袭性感染，另外3例为吻合口气道感染。其中1例受者吻合口发生真菌感染和狭窄，经支气管镜下介入治疗＋泊沙康唑抗真菌治疗效果欠佳，于肺移植术后6个月死于感染。7例受者存活良好，目前肺移植后随访17~47个月，骨髓移植术后35～155个月，肺移植术后1年存活率为87．5％。结论造血干细胞移植术后闭塞性细支气管炎综合征患者行肺移植治疗后并未发现原发病、肿瘤或者BOS等并发症的高风险，且与其他患者比较，存活率相当、甚至更高。肺移植治疗造血干细胞移植后终末期BOS安全、有效。

Objective To investigate the timing and complications of lung transplantation for the treatment of bronchiolitis obliterans syndrome （BOS） after hematopoietic stem cell transplantation. Methods The clinical data of patients with BOS after hematopoietic stem cell transplantation from January 2012 to September 2015 were collected. Results Eight patients with acute BOS after hematopoietic stem cell transplantation were evaluated and received lung transplantation. The recipient age was 30. 88-＋ 12. 12 years （13-47 years）. The lung transplantation was performed 3. 44 ＋- 1.29 years （1-5 years） after BOS. Four patients received bilateral lung transplantation： 2 given single lung transplantation, one given peetus excavatum surgery during the bilateral lung transplantation, and one 1-year-old child given bilateral lobar grafting. Two cases of acute rejection occurred on day 2 and day 9 respectively after transplantation. Four patients experienced early stage aspergillosis infection： one suffering from pulmonary parenchymal invasive infection and the rest 3 cases from anastomotic airway infection. In these 3 cases, one with anastomotic fungal infection and stenosis got poor therapeutic effect by bronchoscopic interventional pluspoosconazole antifungal treatment, and died of lung infection at 6th month after transplantation. Seven patients survived well. The follow-up period ranged from 17 to 47 months after lung transplantation and 35.55 months after bone marrow transplantation. The one-year survival rate after lung transplantation was 87. 5%. Conclusion Patients with BOS after hematopoietic stem cell transplantation do not have a high risk of complications such as primary disease, tumor or BOS after lung transplantation, and the survival rate is comparable or even superior to other patients. Lung transplantation for hematopoietic stem cell transplantation after end-stage BOS is safe and effective.