Wolff D, Reichenberger F, Steiner B, Kahl C, Leithäuser M, Skibbe T, Friedrich T, Terpe H, Helbig W, Freund M
Division of Hematology and Oncology, Department of Internal Medicine, University of Rostock, Germany.
Bone Marrow Transplant. 2002 Feb;29(4):357-60. doi: 10.1038/sj.bmt.1703386.
Sclerodermoid chronic graft-versus-host disease (sGVHD) is a well-known complication in patients with a long history of chronic GVHD. Pulmonary involvement in chronic GVHD presents typically as bronchiolitis obliterans (BO). Pulmonary fibrosis after allogeneic hematopoietic stem cell transplantation (HSCT) is presumed to be caused by the long-term toxicity of the conditioning regimen or the result of lung injury elicited predominantly by viral infections or GVHD. We present two patients with late onset pulmonary fibrosis associated with moderate sGVHD of the skin after HSCT. At the initial diagnosis of chronic GVHD both patients presented with symptoms of interstitial pneumonia. Years later both patients developed moderate to severe interstitial pulmonary fibrosis in association with sGVHD. One patient showed additional clinical and histological signs of BO. While one patient responded to increased immunosuppression including total nodal irradiation (1 Gy), the other patient died due to complications related to pulmonary fibrosis.
硬皮病样慢性移植物抗宿主病（sGVHD）是有慢性移植物抗宿主病（GVHD）病史患者中一种众所周知的并发症。慢性GVHD的肺部受累通常表现为闭塞性细支气管炎（BO）。异基因造血干细胞移植（HSCT）后发生的肺纤维化被认为是预处理方案的长期毒性所致，或是主要由病毒感染或GVHD引发的肺损伤的结果。我们报告了两名在HSCT后出现与中度皮肤sGVHD相关的迟发性肺纤维化患者。在慢性GVHD的初始诊断时，两名患者均表现出间质性肺炎症状。数年后，两名患者均出现与sGVHD相关的中度至重度间质性肺纤维化。一名患者还表现出BO的额外临床和组织学体征。一名患者对包括全淋巴结照射（1 Gy）在内的免疫抑制增加有反应，而另一名患者因与肺纤维化相关的并发症死亡。
