Extremely late-onset chronic graft-versus-host disease presenting with bronchiolitis obliterans and pleuroparenchymal fibroelastosis

Abstract

A 35-year-old woman experienced left back pain after a 2-h flight. She reported coughing and left back pain 1 day later when she presented to our hospital. Chest computed tomography showed pneumothorax of the left lung, bronchiectasis, thickening of the bronchial wall, nodules, and cavity lesions in both lungs. A pulmonary function test revealed obstructive ventilation disorder with normal lung diffusing capacity. She had a history of haematopoietic stem cell transplantation (HSCT) at 2 years and 3 months of age during the second disease remission of acute myeloid leukaemia. She was diagnosed with chronic graft-versus-host disease (cGVHD) presenting with bronchiolitis obliterans (BO) and pleuroparenchymal fibroelastosis (PPFE). To our knowledge, this is the first reported case of BO and PPFE diagnosed more than 30 years after HSCT.

Keywords: bronchiolitis obliterans; graft‐versus‐host disease; haematopoietic stem cell transplantation; late onset; pleuroparenchymal fibroelastosis.