9例儿童异基因造血干细胞移植后闭塞性细支气管炎综合征的临床特征

Clinical characteristics of bronchiolitis obliterans syndrome after allogeneichematopoietic stem cell transplantation in 9 children

目的 分析儿童异基因造血干细胞移植(allo-HSCT)术后并发闭塞性细支气管炎综合征(BOS)的临床特征。方法 收集2014年8月至2021年3月于山东省千佛山医院住院行allo-HSCT后确诊BOS的9例患儿的病历资料,回顾性分析9例患儿的临床症状、胸部高分辨CT、肺功能、诊断以及治疗。结果 9例患儿allo-HSCT术后出现BOS的时间为(9.3±4.4)个月,主要临床表现为干咳、气喘。BOS在胸部高分辨CT中的主要表现为支气管壁增厚、支气管扩张和马赛克征等。肺功能改变以阻塞性通气功能障碍同时伴有弥散功能下降为主。allo-HSCT后BOS的治疗主要以免疫抑制剂和糖皮质激素为主,9例患儿治疗后均好转。结论 BOS是儿童行allo-HSCT治疗后发生在肺部的排异表现,其临床表现、肺功能以及胸部CT表现缺乏特异性。BOS会造成肺部不可逆性损害,移植后患儿需规律行肺功能检查以早诊断、早治疗。

Objective To analyze the clinical characteristics of bronchiolitis obliterans syndrome(BOS) in children who had received allogeneic hematopoietic stem cell transplantation(allo-HSCT). Methods The clinical data of 9 children with BOS after allo-HSCT treated in Shandong Provincial Qianfoshan Hospital during Aug. 2014 and Mar. 2021 were retrospectively analyzed, including the clinical characteristics, chest imaging features, pulmonary function, diagnosis and treatment. Results The onset of BOS after allo-HSCT was(9.3±4.4) months. Cough and wheezing were the main clinical features. The main manifestations of BOS in the high resolution CT(HRCT) were bronchiectasis, bronchial wall thickening and mosaic sign. Pulmonary function was mainly characterized with obstructive ventilation dysfunction and decreased diffusion function. The rnain treatment were immunosuppressive therapy and corticosteroids. All 9 children improved after treatment. Conclusion BOS is a pulmonary rejection manifestation in children after allo-HSCT. There are no specific clinical manifestations, pulmonary function tests and HRCT findings. Children undergoing transplantation need regular lung function examinations for early diagnosis and treatment.