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

Osamu Imataki; Makiko Uemura

doi:10.1002/ccr3.8415

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

Clin Case Rep. 2024 Jan 3;12(1):e8415. doi: 10.1002/ccr3.8415. eCollection 2024 Jan.

Authors

Osamu Imataki¹, Makiko Uemura¹

Affiliation

¹ Division of Hematology, Department of Internal Medicine, Faculty of Medicine Kagawa University Takamatsu Japan.

Abstract

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).

Publication types

Case Reports