Pleuroparenchymal fibroelastosis after haematopoietic stem cell transplantation without graft-versus-host disease findings

Although rare, pleuroparenchymal fibroelastosis (PPFE) is a serious late-onset complication of haematopoietic stem cell transplantation (HSCT). It remains unclear whether graft-versus-host disease (GVHD) is involved in the development of PPFE. We report the case of a patient with PPFE after HSCT. The patient experienced pneumothorax repeatedly despite surgical treatment. A surgical specimen demonstrated PPFE findings, without evidence of GVHD. In this case, development of PPFE was not associated with GVHD, and immunosuppressive therapy did not improve pulmonary function. Surgical biopsy is recommended for precise treatment and elucidation of pathogenesis in each suspected PPFE patient.