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[异基因造血干细胞移植后间质性肺炎并发空气泄漏综合征的泼尼松龙成功治疗]

[Successful treatment with prednisolone for air-leak syndrome following interstitial pneumonia after allogeneic hematopoietic stem cell transplantation].

作者信息

Honda Yuko, Miyaji Ryosuke, Morita Hiromi, Inagaki Jiro, Kusuhara Koichi

机构信息

Department of Pediatrics, University of Occupational and Environmental Health.

出版信息

Rinsho Ketsueki. 2012 Apr;53(4):455-9.

Abstract

A 13-year-old boy with T lymphoblastic leukemia underwent allogeneic bone marrow transplantation from an HLA-matched sibling in the second remission phase. After the dose of cyclosporine (CyA) was reduced, dyspnea appeared on Day 117. CT revealed diffuse interstitial shadows on the bilateral lungs. The results of broncho-alveolar lavage suggested Pneumocystis jirovecii pneumonia. The dose of trimethoprim-sulfamethoxazole was increased, and steroid therapy was started. The symptoms transiently subsided, but exacerbated with a reduction in the steroid dose. On Day 139, mediastinal and subcutaneous emphysema appeared. We considered that non-infectious interstitial pneumonia was primarily involved in the pathogenesis for the following reasons: the boy was negative for β-D-glucan early after onset, and there was a correlation between the steroid-dose reduction and condition. The steroid dose was again increased to 80 mg and the symptoms promptly subsided. When late-onset non-infectious pulmonary complications after hematopoietic stem cell transplantation lead to air-leak syndrome, the mortality rate is very high. However, survival may be achieved by intensifying immunosuppressive therapy in the early stage.

摘要

一名13岁的T淋巴细胞白血病男孩在第二次缓解期接受了来自HLA匹配同胞的异基因骨髓移植。在环孢素(CyA)剂量减少后,第117天出现呼吸困难。CT显示双侧肺部弥漫性间质阴影。支气管肺泡灌洗结果提示耶氏肺孢子菌肺炎。增加了甲氧苄啶-磺胺甲恶唑的剂量,并开始了类固醇治疗。症状暂时缓解,但随着类固醇剂量的减少而加重。第139天,出现纵隔和皮下气肿。我们认为非感染性间质性肺炎主要参与发病机制,原因如下:男孩发病早期β-D-葡聚糖检测为阴性,且类固醇剂量减少与病情之间存在相关性。类固醇剂量再次增加至80mg,症状迅速缓解。当造血干细胞移植后的迟发性非感染性肺部并发症导致空气泄漏综合征时,死亡率非常高。然而,通过在早期强化免疫抑制治疗可能实现存活。

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