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


Authors: Marathe, P. H.; Santibanez, V.; Meyers, P. A.; Padilla, M. L.; Friedman, D. N.
Article Title: Pleuroparenchymal fibroelastosis as a late complication of childhood cancer therapy: A case series
Abstract: Pleuroparenchymal 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. © 2024 Wiley Periodicals LLC.
Keywords: adolescent; adult; child; clinical article; human tissue; preschool child; school child; child, preschool; antibiotic agent; young adult; hydroxyurea; prednisone; case report; cisplatin; doxorubicin; cytarabine; methotrexate; temozolomide; neoplasm; neoplasms; carboplatin; computer assisted tomography; etoposide; lung disease; differential diagnosis; oxygen therapy; exercise; alkylating agent; cyclophosphamide; melphalan; vincristine; hematopoietic stem cell transplantation; pathology; cancer therapy; carmustine; ifosfamide; acute lymphoblastic leukemia; childhood cancer; irinotecan; ewing sarcoma; salbutamol; neuroblastoma; antineoplastic agents, alkylating; survivorship; dactinomycin; asparaginase; daily life activity; interstitial lung disease; thorax radiography; graft survival; isotretinoin; drug therapy; lung function; alkylating agents; lung function test; lung fibrosis; pulmonary fibrosis; therapy; tioguanine; etiology; medical history; adverse event; complication; ipratropium bromide; forced vital capacity; wedge resection; childhood cancer survivor; antifibrotic agent; lung diseases, interstitial; fluticasone; pleuroparenchymal fibroelastosis; humans; prognosis; human; male; female; article; total lung capacity; six minute walk test; pulmonary late effects; tiotropium bromide
Journal Title: Pediatric Blood and Cancer
Volume: 71
Issue: 7
ISSN: 1545-5009
Publisher: Wiley Periodicals, Inc  
Date Published: 2024-07-01
Start Page: e31004
Language: English
DOI: 10.1002/pbc.31004
PUBMED: 38637875
PROVIDER: scopus
DOI/URL:
Notes: Article -- Source: Scopus
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  1. Danielle Novetsky Friedman
    110 Friedman
  2. Paul Meyers
    311 Meyers