Yabe M, Yabe H, Hattori K, Morimoto T, Hinohara T, Takakura I, Shimizu T, Shimamura K, Tang X, Kato S
Department of Pediatrics, Tokai University School of Medicine, Isehara, Japan.
Bone Marrow Transplant. 1997 Feb;19(4):389-92. doi: 10.1038/sj.bmt.1700674.
Chronic restrictive lung disease in a 9-year-old boy with dyskeratosis congenita (DC) 7 years after allogeneic bone marrow transplantation (BMT) is described. When he was 1 year and 10 months old, severe aplastic anemia developed. He received a marrow transplant from his HLA serologically identical, but HLA-DP mismatched brother. He developed grade II acute graft-versus-host disease (GVHD) and thereafter chronic GVHD of progressive type, and was treated with both prednisolone and azathioprine resulting in clinical improvement. Thereafter he complained of dyspnea, and bilateral noncircumscribed interstitial shadows on chest CT scan were present. His pulmonary function showed restrictive changes. Prednisolone was not effective and he died of respiratory failure. Post-mortem examination confirmed interstitial fibrosis, lymphocytic infiltration of the bronchioles and alveoli with luminal fibrosis. There was no evidence of chronic GVHD in the skin and the liver. These findings raise the possibility that this pulmonary complication was associated with DC itself.
本文描述了一名9岁男孩在异基因骨髓移植（BMT）7年后发生慢性限制性肺病，该男孩患有先天性角化不良（DC）。他1岁10个月大时出现严重再生障碍性贫血。他接受了来自其HLA血清学相同但HLA-DP不匹配的哥哥的骨髓移植。他发生了II级急性移植物抗宿主病（GVHD），此后发展为进行性慢性GVHD，并接受泼尼松龙和硫唑嘌呤治疗，临床症状有所改善。此后他出现呼吸困难，胸部CT扫描显示双侧边界不清的间质性阴影。他的肺功能显示出限制性改变。泼尼松龙治疗无效，他死于呼吸衰竭。尸检证实存在间质纤维化、细支气管和肺泡的淋巴细胞浸润以及管腔纤维化。皮肤和肝脏没有慢性GVHD的证据。这些发现提示这种肺部并发症可能与DC本身有关。
