Pleuroparenchymal fibroelastosis as a late complication of childhood cancer therapy: A case series

Abstract

AbstractPleuroparenchymal fibroelastosis (PPFE) is a rare interstitial pneumonia with distinct clinicopathologic features. It has been associated with exposure to hematopoietic stem cell transplantation (HSCT) and classical alkylating agents. Here, we highlight PPFE as a late complication of childhood cancer therapy by describing the cases of four survivors of childhood cancer with a diagnosis of treatment‐related PPFE. All patients received high‐dose alkylating agents. PPFE should be considered in the differential diagnosis of restrictive lung disease in patients with history of exposure to alkylating agents or HSCT. Development of PPFE‐specific, noninvasive diagnostic tools and disease‐modifying therapies will clinically benefit these patients.