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

Overview

Journal Respir Med Case Rep

Date 2022 Mar 22

PMID 35313561

Authors

Hajime Kasai

Jiro Terada

Jun Nagata

Keiko Yamamoto

Shunya Shiohira

Atsuko Tomikawa

Nao Tamura

Emiri Yamamoto

Yuzuru Ikehara

Takuji Suzuki

Affiliations

Soon will be listed here.

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.

References

Higo H, Miyahara N, Taniguchi A, Maeda Y, Kiura K . Cause of pleuroparenchymal fibroelastosis following allogeneic hematopoietic stem cell transplantation. Respir Investig. 2019; 57(4):321-324. DOI: 10.1016/j.resinv.2019.04.003. View

Bondeelle L, Gras J, Michonneau D, Houdouin V, Hermet E, Blin N . Pleuroparenchymal fibroelastosis after allogeneic hematopoietic stem cell transplantation. Bone Marrow Transplant. 2019; 55(5):982-986. DOI: 10.1038/s41409-019-0636-8. View

Mariani F, Gatti B, Rocca A, Bonifazi F, Cavazza A, Fanti S . Pleuroparenchymal fibroelastosis: the prevalence of secondary forms in hematopoietic stem cell and lung transplantation recipients. Diagn Interv Radiol. 2016; 22(5):400-6. PMC: 5019843. DOI: 10.5152/dir.2016.15516. View

Watanabe S, Waseda Y, Takato H, Matsunuma R, Johkoh T, Egashira R . Pleuroparenchymal fibroelastosis: Distinct pulmonary physiological features in nine patients. Respir Investig. 2015; 53(4):149-55. DOI: 10.1016/j.resinv.2015.02.003. View

Yoshida Y, Nagata N, Tsuruta N, Kitasato Y, Wakamatsu K, Yoshimi M . Heterogeneous clinical features in patients with pulmonary fibrosis showing histology of pleuroparenchymal fibroelastosis. Respir Investig. 2016; 54(3):162-9. DOI: 10.1016/j.resinv.2015.11.002. View

Tanizawa K, Handa T, Kubo T, Chen-Yoshikawa T, Aoyama A, Motoyama H . Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study. Respir Res. 2018; 19(1):162. PMC: 6117972. DOI: 10.1186/s12931-018-0860-6. View

Kumar S, Tefferi A . Spontaneous pneumomediastinum and subcutaneous emphysema complicating bronchiolitis obliterans after allogeneic bone marrow transplantation--case report and review of literature. Ann Hematol. 2001; 80(7):430-5. DOI: 10.1007/s002770100301. View

Namkoong H, Ishii M, Mori T, Sugiura H, Tasaka S, Sakurai M . Clinical and radiological characteristics of patients with late-onset severe restrictive lung defect after hematopoietic stem cell transplantation. BMC Pulm Med. 2017; 17(1):123. PMC: 5590140. DOI: 10.1186/s12890-017-0466-7. View

Takeuchi Y, Miyagawa-Hayashino A, Chen F, Kubo T, Handa T, Date H . Pleuroparenchymal fibroelastosis and non-specific interstitial pneumonia: frequent pulmonary sequelae of haematopoietic stem cell transplantation. Histopathology. 2014; 66(4):536-44. DOI: 10.1111/his.12553. View

10.

Ishii T, Bandoh S, Kanaji N, Tadokoro A, Watanabe N, Imataki O . Air-leak Syndrome by Pleuroparenchymal Fibroelastosis after Bone Marrow Transplantation. Intern Med. 2016; 55(2):105-11. DOI: 10.2169/internalmedicine.55.4539. View