Clinical and radiological characteristics of patients with late-onset severe restrictive lung defect after hematopoietic stem cell transplantation

Ho Namkoong, Makoto Ishii, Takehiko Mori, Hiroaki Sugiura, Sadatomo Tasaka, Masatoshi Sakurai, Yuya Koda, Jun Kato, Naoki Hasegawa, Shinichiro Okamoto, Tomoko Betsuyaku

Research output: Contribution to journalArticle

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Abstract

Background: Late-onset noninfectious pulmonary complications (LONIPCs), which occur more than 3months after allogeneic hematopoietic stem cell transplantation (HSCT), are major causes of morbidity and mortality after transplantation. Among LONIPCs, we occasionally treat patients with late-onset severe restrictive lung defect after HSCT; however, its clinical features have not been fully elucidated. Methods: A retrospective chart review of a single center on cases of late-onset severe restrictive lung defect after HSCT was performed. Among 453 patients who survived longer than 100days after allogeneic HSCT with evaluable spirometry data, 12 patients (2.6%) developed late-onset severe restrictive lung defect (i.e., vital capacity percent of predicted less than 60%). Results: Median duration from transplantation to diagnosis of late-onset severe restrictive lung defect cases was 44.5months. Major computed tomography (CT) finding was pleuroparenchymal thickening with volume loss, an evidence of fibrosis, predominantly in upper lobes (n=7), which was consistent with pleuroparenchymal fibroelastosis. The remaining patients showed unclassifiable interstitial pneumonia pattern (n=2) and airway-predominant pattern (n=3). The diffusing capacity for carbon oxide tended to decrease, while the residual volume/total lung capacity ratio tended to increase after HSCT. Of 12 patients, 8 patients died and the median month from diagnosis to death was 33.5months. Seven patients died of pulmonary or systemic infection, and one patient died due to relapse of the primary disease. Conclusion: Severe restrictive lung defect could develop in selected cases in the late-phase after HSCT and could be a unique clinical entity with specific radiographical findings.

Original languageEnglish
Article number123
JournalBMC Pulmonary Medicine
Volume17
Issue number1
DOIs
Publication statusPublished - 2017 Sep 7

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Hematopoietic Stem Cell Transplantation
Lung
Transplantation
Total Lung Capacity
Residual Volume
Delayed Diagnosis
Spirometry
Interstitial Lung Diseases
Vital Capacity
Oxides
Fibrosis
Carbon
Tomography
Morbidity
Recurrence
Mortality
Infection

Keywords

  • Hematopoietic stem cell transplantation
  • Idiopathic pneumonia syndrome
  • Late-onset noninfectious pulmonary complications
  • Pleuroparenchymal fibroelastosis

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Clinical and radiological characteristics of patients with late-onset severe restrictive lung defect after hematopoietic stem cell transplantation. / Namkoong, Ho; Ishii, Makoto; Mori, Takehiko; Sugiura, Hiroaki; Tasaka, Sadatomo; Sakurai, Masatoshi; Koda, Yuya; Kato, Jun; Hasegawa, Naoki; Okamoto, Shinichiro; Betsuyaku, Tomoko.

In: BMC Pulmonary Medicine, Vol. 17, No. 1, 123, 07.09.2017.

Research output: Contribution to journalArticle

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abstract = "Background: Late-onset noninfectious pulmonary complications (LONIPCs), which occur more than 3months after allogeneic hematopoietic stem cell transplantation (HSCT), are major causes of morbidity and mortality after transplantation. Among LONIPCs, we occasionally treat patients with late-onset severe restrictive lung defect after HSCT; however, its clinical features have not been fully elucidated. Methods: A retrospective chart review of a single center on cases of late-onset severe restrictive lung defect after HSCT was performed. Among 453 patients who survived longer than 100days after allogeneic HSCT with evaluable spirometry data, 12 patients (2.6{\%}) developed late-onset severe restrictive lung defect (i.e., vital capacity percent of predicted less than 60{\%}). Results: Median duration from transplantation to diagnosis of late-onset severe restrictive lung defect cases was 44.5months. Major computed tomography (CT) finding was pleuroparenchymal thickening with volume loss, an evidence of fibrosis, predominantly in upper lobes (n=7), which was consistent with pleuroparenchymal fibroelastosis. The remaining patients showed unclassifiable interstitial pneumonia pattern (n=2) and airway-predominant pattern (n=3). The diffusing capacity for carbon oxide tended to decrease, while the residual volume/total lung capacity ratio tended to increase after HSCT. Of 12 patients, 8 patients died and the median month from diagnosis to death was 33.5months. Seven patients died of pulmonary or systemic infection, and one patient died due to relapse of the primary disease. Conclusion: Severe restrictive lung defect could develop in selected cases in the late-phase after HSCT and could be a unique clinical entity with specific radiographical findings.",
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AU - Namkoong, Ho

AU - Ishii, Makoto

AU - Mori, Takehiko

AU - Sugiura, Hiroaki

AU - Tasaka, Sadatomo

AU - Sakurai, Masatoshi

AU - Koda, Yuya

AU - Kato, Jun

AU - Hasegawa, Naoki

AU - Okamoto, Shinichiro

AU - Betsuyaku, Tomoko

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N2 - Background: Late-onset noninfectious pulmonary complications (LONIPCs), which occur more than 3months after allogeneic hematopoietic stem cell transplantation (HSCT), are major causes of morbidity and mortality after transplantation. Among LONIPCs, we occasionally treat patients with late-onset severe restrictive lung defect after HSCT; however, its clinical features have not been fully elucidated. Methods: A retrospective chart review of a single center on cases of late-onset severe restrictive lung defect after HSCT was performed. Among 453 patients who survived longer than 100days after allogeneic HSCT with evaluable spirometry data, 12 patients (2.6%) developed late-onset severe restrictive lung defect (i.e., vital capacity percent of predicted less than 60%). Results: Median duration from transplantation to diagnosis of late-onset severe restrictive lung defect cases was 44.5months. Major computed tomography (CT) finding was pleuroparenchymal thickening with volume loss, an evidence of fibrosis, predominantly in upper lobes (n=7), which was consistent with pleuroparenchymal fibroelastosis. The remaining patients showed unclassifiable interstitial pneumonia pattern (n=2) and airway-predominant pattern (n=3). The diffusing capacity for carbon oxide tended to decrease, while the residual volume/total lung capacity ratio tended to increase after HSCT. Of 12 patients, 8 patients died and the median month from diagnosis to death was 33.5months. Seven patients died of pulmonary or systemic infection, and one patient died due to relapse of the primary disease. Conclusion: Severe restrictive lung defect could develop in selected cases in the late-phase after HSCT and could be a unique clinical entity with specific radiographical findings.

AB - Background: Late-onset noninfectious pulmonary complications (LONIPCs), which occur more than 3months after allogeneic hematopoietic stem cell transplantation (HSCT), are major causes of morbidity and mortality after transplantation. Among LONIPCs, we occasionally treat patients with late-onset severe restrictive lung defect after HSCT; however, its clinical features have not been fully elucidated. Methods: A retrospective chart review of a single center on cases of late-onset severe restrictive lung defect after HSCT was performed. Among 453 patients who survived longer than 100days after allogeneic HSCT with evaluable spirometry data, 12 patients (2.6%) developed late-onset severe restrictive lung defect (i.e., vital capacity percent of predicted less than 60%). Results: Median duration from transplantation to diagnosis of late-onset severe restrictive lung defect cases was 44.5months. Major computed tomography (CT) finding was pleuroparenchymal thickening with volume loss, an evidence of fibrosis, predominantly in upper lobes (n=7), which was consistent with pleuroparenchymal fibroelastosis. The remaining patients showed unclassifiable interstitial pneumonia pattern (n=2) and airway-predominant pattern (n=3). The diffusing capacity for carbon oxide tended to decrease, while the residual volume/total lung capacity ratio tended to increase after HSCT. Of 12 patients, 8 patients died and the median month from diagnosis to death was 33.5months. Seven patients died of pulmonary or systemic infection, and one patient died due to relapse of the primary disease. Conclusion: Severe restrictive lung defect could develop in selected cases in the late-phase after HSCT and could be a unique clinical entity with specific radiographical findings.

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KW - Pleuroparenchymal fibroelastosis

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