异基因造血干细胞移植后迟发性非感染肺部并发症分析

目的 分析异基因造血干细胞移植（allo-HSCT）后迟发性非感染肺部并发症（LONIPC）的临床特征、治疗和转归。方法对17例确诊恶性血液病接受allo-HSCT且生存期超过3个月确诊为LONIPC的患者临床资料进行回顾性分析。结果LONIPC发病率为17．7％,起病中位时间为移植后6．5个月（3～13．5个月）,7例出现于环孢素A（CSA）快速减量过程中。15例发病时合并有慢性移植物抗宿主病（cGVHD）。发病初期均表现为不同程度的呼吸困难、干咳,5例有低、中度发热。肺部CT特征：毛玻璃样改变,不规则片状实变、条索、结节状影等。肺组织病理特征：问质内淋巴细胞、浆细胞及组织细胞浸润,肺间质纤维组织增生,肺泡闭塞。皮质激素和辅以CSA治疗有效率为70．6％,早期治疗疗效优于晚期治疗。LONIPC相关死亡率为35．3％,迁延不愈患者CT提示肺组织纤维化。结论LONIPC的临床表现及影像学无特异性,诊断需结合肺功能检查、肺活检病理,并排除原体感染,其中肺组织病理检查对诊断有重要意义;LONIPC可能是cGVHD在肺组织中的特殊表现,cGVHD是LONIPC诊断的重要佐证;早期皮质激素治疗及维持治疗时间充分,对LONIPC的缓解及防治肺部纤维化等后遗症有重要意义。

Non-infectious pulmonary complications after allogeneic hematopoietic stem cell transplantation: analysis of 17 cases

Objective To analyze the clinical feature, cause, treatment and outcome of late onset non-infectious pulmonary complications (LONIPC) after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Methods The clinical data of 17 patients with malignant hematological diseases who survived for at least 3 months and suffered from LONIPC after allo- HSCT were retrospectively analyzed. Results The incidence of LONIPC was 17.7% in allo-HSCT recipients. The median of onset of respiratory symptoms was 6.5 months (3~13.5 months). Seven patients came down with LONIPC during fast tapering of cyclosporine A prophylaxis and 15 patients had already chronic graft versus host disease (cGVHD) before the occurrence of respiratory symptoms. The initial symptoms were non-productive cough and dyspnoea in all patients; five of them had low grade or moderate fever. CT scans revealed patchy ground-glass opacities, irregular patchy consolidation, band-like opacities, and micronodular densities. Histological examination of transbronchial biopsy showed infiltration of lymphocyte and monocytes in interstitium, peribronchiolar fibrosis and alveoli pulmonis obliteration. The response rate of corticosteroids in addition to cyclosporine therapy was 70.6%. Treatment beginning at the early stage was more effective than that beginning late. The mortality rate of LONIPC was 35.3% .Chest CT scanning showed lung fibrosis in the patients with protracted LONIPC. Conclusion The clinical manifestations and radiological changes of LONIPC are non-specific. The diagnosis is made by combination of functional and histological examinations and exclusion of pathogen infection. Examination of transbronchial biopsy is of significance for the diagnosis. LONIPC may be considered as pathognomonic of cGVHD in the lung of patients after allo-HSCT; and cGVHD should be regarded as a useful diagnostic proof for LONIPC. Earlier treatment with corticosteroids and maintenance treatment may result in improved survival and decrease of the fibrotic residue.