Abstract
Late-onset noninfectious pulmonary complication after allogenic hematopoietic stem cell transplantation is an important contributing factor associated with high rate morbidity and mortality. We report a case with pleuroparenchymal fibroelastosis (PPFE) occurred after allogenic bone marrow transplantation. The onset was infiltrative shadows in upper lobes, and the haziness spread gradually throughout the lungs with recurrent episodes of pneumothorax in both lungs. Progressive respiratory failure in course of adrenocortical steroid administration eventually caused death. Histological examination in general autopsy showed patchy subpleural fibrosis predominantly distributed in the upper lobes with substantial density rise of elastic fibers in the subpleural area, consistent with the diagnosis of PPFE. PPFE after allogenic transplantation has been seldom reported to date, but it is one of the most important histological components of late-onset noninfectious pulmonary complication after allogenic transplantation characterized by recurrent pneumothorax. Retrospective analysis in our case indicates early diagnosis may be possible by histological evaluation of elastic fibers in lung specimen when pneumothorax is treated surgically. This case suspects that it is important for hematologist and pathologist to aware this progressive disease along with information of histological characteristics, therefore, leading to the establishment of therapeutic strategies and the improvement of poor prognosis.
Similar content being viewed by others
References
Sakaida E, Nakaseko C, Harima A, Yokota A, Cho R, Saito Y, et al. Late-onset noninfectious pulmonary complications after allogeneic stem cell transplantation are significantly associated with chronic graft-versus-host disease and with the graft-versus-leukemia effect. Blood. 2003;102:4236–42.
Patriarca F, Skert C, Sperotto A, Damiani D, Cerno M, Geromin A, et al. Incidence, outcome, and risk factors of late-onset noninfectious pulmonary complications after unrelated donor stem cell transplantation. Bone Marrow Transplant. 2004;33:751–8.
Frankel SK, Cool CD, Lynch DA, Brown KK. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest. 2004;126:2007–13.
von der Thüsen JH, Hansell DM, Tominaga M, Veys PA, Ashworth MT, Owens CM, et al. Pleuroparenchymal fibroelastosis in patients with pulmonary disease secondary to bone marrow transplantation. Mod Pathol. 2011;24:1633–9.
Fujikura Y, Kanoh S, Kouzaki Y, Hara Y, Matsubara O, Kawana A. Pleuroparenchymal fibroelastosis as a series of airway complications associated with chronic graft-versus-host disease following allogeneic bone marrow transplantation. Intern Med. 2014;53:43–6.
Amitani R, Niimi A, Kuze F. Idiopathic pulmonary upper lobe fibrosis [in Japanese]. Kokyu. 1992;11:693–9.
Rosenbaum JN, Butt YM, Johnson KA, Meyer K, Batra K, Kanne JP, et al. Pleuroparenchymal fibroelastosis: a pattern of chronic lung injury. Hum Pathol. 2015;46:137–46.
Takeuchi Y, Miyagawa-Hayashino A, Chen F, Kubo T, Handa T, Date H, et al. Pleuroparenchymal fibroelastosis and non-specific interstitial pneumonia: frequent pulmonary sequelae of haematopoietic stem cell transplantation. Histopathology. 2015;66:536–44.
Oda T, Ogura T, Kitamura H, Hagiwara E, Baba T, Enomoto Y, et al. Distinct characteristics of pleuroparenchymal fibroelastosis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis. Chest. 2014;146:1248–55.
Sakai R, Kanamori H, Nakaseko C, Yoshiba F, Fujimaki K, Sakura T, et al. Air-leak syndrome following allo-SCT in adult patients: report from the Kanto Study Group for Cell Therapy in Japan. Bone Marrow Transplant. 2011;46:379–84.
Yamanoha A, Nagasaki A, Nakachi S, Kinjo S, Takasu N. Air-leak syndrome associated with bronchiolitis obliterans after allogeneic peripheral blood stem cell transplantation. Int J Hematol. 2007;85:95–6.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
All authors declare no conflict of interest.
About this article
Cite this article
Matsui, T., Maeda, T., Kida, T. et al. Pleuroparenchymal fibroelastosis after allogenic hematopoietic stem cell transplantation: important histological component of late-onset noninfectious pulmonary complication accompanied with recurrent pneumothorax. Int J Hematol 104, 525–530 (2016). https://doi.org/10.1007/s12185-016-2038-7
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12185-016-2038-7