A case of thoracic air leak syndrome with pleural parenchymal fibroelastosis after treatment for hematologic malignancy while awaiting lung transplantation: Imaging and pathological findings of rapid loss in lung volume

Abstract

We report the case of a 29-year-old man who underwent umbilical cord blood transplantation for chronic myelogenous leukemia 14 years previously. He was diagnosed with secondary pleuroparenchymal fibroelastosis (sPPFE) following treatment for hematologic malignancies (sPPFE after HM-Tx) 2.5 years ago. On computed tomography, pleural thickening in the upper lobe, lung volume loss, and recurrent bilateral pneumothorax were detected. Although he waited for cadaveric lung transplantation (LTx) for 1.5 years, his respiratory failure worsened, and he died. Pathological autopsy and clinical course indicated sPPFE. After diagnosing sPPFE after HM-Tx, the timing for deciding LTx is critical, especially when pneumothorax recurs.

Keywords

• Pleuroparenchymal fibroelastosis
• Hematopoietic stem cell transplantation
• Lung transplantation
• Pneumothorax
• Thoracic air-leak syndrome