同種骨髄移植後の反復する気胸を契機に早期発見することができた移植片対宿主病変

Graft-versus-host Disease Detected Early Due to Repeated Pneumothorax after Bone Marrow Transplantation

骨髄移植後の慢性肺疾患は予後不良な肺合併症として注目されている．われわれは骨髄移植後11年経過し，相次いで左右に発症した気胸に対する手術時の摘出標本において，組織学的に胸膜肺実質線維弾性症（PPFE）を認め，慢性肺移植片対宿主病（GVHD）の発症を疑った1例を経験したので，報告する．

A 15-year-old boy who presented recurrent bilateral pneumothoraces after allogenic bone marrow transplantation for the treatment of myelodysplastic syndrome is presented. We performed bulla resection under the thoracoscopic surgery for three times. Pathological examination revealed irregular fibrous thickening of the visceral pleura and alveolar fibrosis, consistent with a diagnosis of pleuroparenchymal fibroelastosis （PPFE）. Also the findings of bronchiolitis obliterans （BO） was noted and chronic graft-versus-host disease （GVHD） was strongly suggested. Twenty-five months after the operation, bilateral living-donor lobar lung transplantation was performed and the diagnosis of GVHD was established.