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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:间质内淋巴细胞、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,并排除病原体感染,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。
Objective To analyze the clinical characteristics, treatment and prognosis of delayed noninfectious pulmonary complications (LONIPC) after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Methods A retrospective analysis was performed on the clinical data of 17 patients diagnosed as LONIPC with allo-HSCT diagnosed with hematologic malignancies over 3 months. Results The incidence of LONIPC was 17.7%. The median time to onset was 6.5 months (3-13.5 months) after transplantation, and 7 cases occurred during the rapid reduction of CSA. Fifteen cases were associated with chronic graft-versus-host disease (cGVHD). Early onset showed varying degrees of dyspnea, dry cough, 5 cases have low and moderate fever. Pulmonary CT features: frosted glass-like changes, irregular lamellar consolidation, cords, nodular shadow. Pathological features of lung tissue: interstitial lymphocytes, plasma cells and tissue infiltration, pulmonary interstitial fibrosis, alveolar occlusion. Corticosteroids and supplemented with CSA treatment efficiency was 70.6%, early treatment is better than the late treatment. The LONIPC-related mortality rate was 35.3%. CT in patients with uncontinuity was associated with lung fibrosis. Conclusion The clinical manifestations and imaging of LONIPC are nonspecific. The diagnosis should be combined with pulmonary function tests and lung biopsy pathology, and exclude pathogen infection, of which lung pathological examination is of great significance for diagnosis; LONIPC may be a special manifestation of cGVHD in lung tissue , cGVHD is an important evidence for the diagnosis of LONIPC; early corticosteroid therapy and maintenance of treatment time, the ease of LONIPC and prevention and treatment of sequelae such as pulmonary fibrosis and other important.