Oo Zun Pwint, Bychkov Andrey, Zaizen Yoshiaki, Yamasue Mari, Kadota Jun-Ichi, Fukuoka Junya
Department of Pathology, Nagasaki University Hospital, Nagasaki, Japan.
Department of Pathology, University of Medicine, Mandalay, Myanmar.
Respir Med Case Rep. 2019 Feb 4;26:244-247. doi: 10.1016/j.rmcr.2019.02.001. eCollection 2019.
Pleuroparenchymal fibroelastosis (PPFE) is a newly described entity of interstitial lung disease, which has been recently recognized as a rare complication of bone marrow transplantation. We report a case of 30-year-old man who developed a unique combination of pleuroparenchymal fibroelastosis with cellular and fibrotic non-specific interstitial pneumonia (NSIP) and bronchiolitis obliterans (BO) sixteen years after hematopoietic stem cell transplantation. Histological examination revealed almost exclusive infiltration of CD3-positive T lymphocytes associated with lymphoepithelial lesions and multi-focal denudation of covering epithelial cells in all components. This case suggests PPFE, NSIP, and BO might be conditions of the same spectrum, pathogenetically related to chronic graft-versus-host disease. Immunostaining for CD3 and CD20 in transbronchial lung biopsies may be helpful for identifying graft-versus-host-driven interstitial lung disease.
胸膜实质纤维弹性组织增生症（PPFE）是一种新描述的间质性肺疾病实体，最近被认为是骨髓移植的一种罕见并发症。我们报告一例30岁男性，在造血干细胞移植16年后出现了胸膜实质纤维弹性组织增生症合并细胞性和纤维化非特异性间质性肺炎（NSIP）及闭塞性细支气管炎（BO）的独特组合。组织学检查显示，所有成分中几乎均有CD3阳性T淋巴细胞的浸润，并伴有淋巴上皮病变和覆盖上皮细胞的多灶性剥脱。该病例提示PPFE、NSIP和BO可能属于同一谱系疾病，在发病机制上与慢性移植物抗宿主病相关。经支气管肺活检中CD3和CD20的免疫染色可能有助于识别移植物抗宿主驱动的间质性肺疾病。
