Journal
INTERNAL MEDICINE
Volume 53, Issue 1, Pages 43-46Publisher
JAPAN SOC INTERNAL MEDICINE
DOI: 10.2169/internalmedicine.53.1124
Keywords
allogeneic bone marrow transplantation; bronchiolitis obliterans; pleuroparenchymal fibroelastosis; chronic graft-versus-host disease; idiopathic pulmonary upper lobe fibrosis
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We herein report the case of a 31-year-old woman who presented with bilateral upper lobe volume loss and pleural irregularities with hilar retraction. She had undergone allogeneic bone marrow transplantation (BMT) for the treatment of acute lymphoblastic leukemia nine years earlier. A surgical lung biopsy showed pleural thickening and subpleural alveolar collapse and fibrosis, consistent with a diagnosis of pleuroparenchymal fibroelastosis (PPFE). Antecedent sicca syndrome and the absence of other causes of fibroelastosis suggested that these abnormalities were associated with chronic graft-versus-host disease (cGVHD). PPFE as a late, noninfectious complication is rare; however, the present case suggests a new class of BMT-related pulmonary complications associated with cGVHD.
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