Aggressive progressive pleuroparenchymal fibroelastosis developed long after allogenic hematopoietic stem cell transplantation

Abstract

Key Clinical Message The typical pleuroparenchymal fibroelastosis (PPFE) after hematopoietic stem cell transplantation (HSCT) is characterized by late‐onset progressive pulmonary complication within 5 years. PPFE shows a poor prognosis without definitive standard care other than lung transplantation. Our case indicated an over 20 years late‐onset and aggressively exacerbating PPFE.

Keywords

• hematopoietic stem cell transplantation (HSCT)
• late‐onset noninfectious pulmonary complication (LONIPC)
• pleuroparenchymal fibroelastosis (PPFE)