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Combination of pleuroparenchymal fibroelastosis with non-specific interstitial pneumonia and bronchiolitis obliterans as a complication of hematopoietic stem cell transplantation - Clues to a potential mechanism.

ABSTRACT: Pleuroparenchymal fibroelastosis (PPFE) is a newly described entity of interstitial lung disease, which has been recently recognized as a rare complication of bone marrow transplantation. We report a case of 30-year-old man who developed a unique combination of pleuroparenchymal fibroelastosis with cellular and fibrotic non-specific interstitial pneumonia (NSIP) and bronchiolitis obliterans (BO) sixteen years after hematopoietic stem cell transplantation. Histological examination revealed almost exclusive infiltration of CD3-positive T lymphocytes associated with lymphoepithelial lesions and multi-focal denudation of covering epithelial cells in all components. This case suggests PPFE, NSIP, and BO might be conditions of the same spectrum, pathogenetically related to chronic graft-versus-host disease. Immunostaining for CD3 and CD20 in transbronchial lung biopsies may be helpful for identifying graft-versus-host-driven interstitial lung disease.

SUBMITTER: Oo ZP

PROVIDER: S-EPMC6370562 | biostudies-other | 2019

REPOSITORIES: biostudies-other

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Combination of pleuroparenchymal fibroelastosis with non-specific interstitial pneumonia and bronchiolitis obliterans as a complication of hematopoietic stem cell transplantation - Clues to a potential mechanism.

Oo Zun Pwint ZP Bychkov Andrey A Zaizen Yoshiaki Y Yamasue Mari M Kadota Jun-Ichi JI Fukuoka Junya J

Respiratory medicine case reports 20190204

Pleuroparenchymal fibroelastosis (PPFE) is a newly described entity of interstitial lung disease, which has been recently recognized as a rare complication of bone marrow transplantation. We report a case of 30-year-old man who developed a unique combination of pleuroparenchymal fibroelastosis with cellular and fibrotic non-specific interstitial pneumonia (NSIP) and bronchiolitis obliterans (BO) sixteen years after hematopoietic stem cell transplantation. Histological examination revealed almost ...[more]

PMID: 30792952

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Project description:BackgroundBronchiolitis obliterans (BO) is one of the most common late non-infectious pulmonary complications after hematopoietic stem cell transplantation (HSCT). Lung transplantation (LT) is the only cure for patients with end-stage BO, but the overall efficacy is rarely reported. Our study aims to conclude and elucidate the clinical experience of our single center and provide a reference for the current selection of treatment.MethodsWe retrospectively analyzed the medical records of six patients with post-HSCT BO who received LT in our center from 2015 to 2019. The collected information included demographic data, surgery-related conditions, and postoperative follow-up data, which covered blood tests, infection status assessment, lung function assessment, anesthesia assessment, function assessment of other organs and so on. All patients were regularly followed up after discharge, which in the first year, was performed every 3 months. Over the next 2 years, patients were assessed every 6 months, and after 3 years, the frequency was once annually.ResultsThe mean age of patients at LT time was 28±13 years, with an interval of 72±48 months from HSCT. All patients developed hypercapnia with an average carbon dioxide partial pressure (pCO2) of 71.1±20.8 mmHg. Preoperative pulmonary function tests showed the mean actual forced expiratory volume in 1 second (FEV1) was 16.7%±5.9% of the predicted value in four patients. After assessment, four patients adopted sequential bilateral LT and two adopted right-sided LT. Due to hemodynamic instability, five patients adopted intraoperative assistance of extracorporeal membrane oxygenation (ECMO). One patient died of septic shock 9 days after surgery, and the other five survived healthy for 53±23 months. The actual value of FEV1 at 3 months postoperatively accounted for 57.9%±15.3% of the predicted value. No patients had recurrence of BO.ConclusionsLT may be a treatment worthy of consideration in patients with post-HSCT end-stage BO because it can improve lung function, quality of life and prolong survival of these selected patients.

Dataset Information

Combination of pleuroparenchymal fibroelastosis with non-specific interstitial pneumonia and bronchiolitis obliterans as a complication of hematopoietic stem cell transplantation - Clues to a potential mechanism.

Publications

Combination of pleuroparenchymal fibroelastosis with non-specific interstitial pneumonia and bronchiolitis obliterans as a complication of hematopoietic stem cell transplantation - Clues to a potential mechanism.

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