Radhakrishnan Sabarinath Venniyil, Hildebrandt Gerhard C
Division of Hematology and Hematologic Malignancies, University of Utah, Huntsman Cancer Institute, 2000 Circle of Hope, Salt Lake City, 84112-5550, UT, USA,
Curr Hematol Malig Rep. 2015 Mar;10(1):8-17. doi: 10.1007/s11899-014-0244-z.
Noninfectious pulmonary toxicity after allogeneic hematopoietic stem cell transplantation (allo-HSCT) causes significant morbidity and mortality. Main presentations are idiopathic pneumonia syndrome (IPS) in the acute setting and bronchiolitis obliterans syndrome (BOS) and cryptogenic organizing pneumonia (COP) at later time point. While COP responds well to corticosteroids, IPS and BOS often are treatment refractory. IPS, in most cases, is rapidly fatal, whereas BOS progresses over time, resulting in chronic respiratory failure, impaired quality of life, and eventually, death. Standard second-line treatments are currently lacking, and current approaches, such as augmented T cell-directed immunosuppression, B cell depletion, TNF blockade, extracorporeal photopheresis, and tyroskine kinase inhibitor therapy, are unsatisfactory with responses in only a subset of patients. Better understanding of underlying pathophysiology hopefully results in the identification of future targets for preventive and therapeutic strategies along with an emphasis on currently underutilized rehabilitative and supportive measures.
异基因造血干细胞移植(allo-HSCT)后的非感染性肺部毒性会导致显著的发病率和死亡率。主要表现为急性情况下的特发性肺炎综合征(IPS)以及后期的闭塞性细支气管炎综合征(BOS)和隐源性机化性肺炎(COP)。虽然COP对皮质类固醇反应良好,但IPS和BOS通常对治疗难治。在大多数情况下,IPS会迅速致命,而BOS会随着时间推移而进展,导致慢性呼吸衰竭、生活质量受损,并最终导致死亡。目前缺乏标准的二线治疗方法,而目前的方法,如增强T细胞导向的免疫抑制、B细胞清除、TNF阻断、体外光化学疗法和酪氨酸激酶抑制剂治疗,仅在部分患者中有反应,并不令人满意。更好地理解潜在的病理生理学有望促成未来预防和治疗策略靶点的确定,同时强调目前未充分利用的康复和支持措施。