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Published on: February 14, 2022
Pulmonary function in long-term survivors of pediatric hematopoietic cell transplantation
Paul A Hoffmeister1, David K Madtes, Barry E Storer
1Clinical Research Division, Fred Hutchinson Cancer Research Center, Department of Medicine, University of Washington Medical School, Seattle, Washington 98109, USA.
Insights
Pulmonary dysfunction affects over half of pediatric hematopoietic cell transplant (HCT) survivors long-term. Early detection through regular pulmonary function testing (PFT) is crucial for managing lung complications after HCT.
Area of Science:
- Pediatric Hematology
- Pulmonology
- Transplant Medicine
Background:
- Pediatric hematopoietic cell transplant (HCT) survivors face long-term health challenges.
- Pulmonary complications are a significant concern in this population.
- Identifying risk factors for pulmonary dysfunction is essential for improved patient outcomes.
Purpose of the Study:
- To determine the prevalence of pulmonary dysfunction in pediatric HCT survivors.
- To identify risk factors associated with restrictive lung disease (RLD) and obstructive lung disease (OLD) post-HCT.
Main Methods:
- Cross-sectional study involving pediatric HCT survivors at least 5 years post-transplant.
- Pulmonary function testing (PFT) was conducted to assess lung function.
- Multivariate analysis was used to identify risk factors for RLD and OLD.
Main Results:
- 55% of participants had pulmonary dysfunction (RLD, OLD, or mixed).
- Moderate-to-severe impairment was noted in 45% of those with RLD or OLD.
- Risk factors for RLD included specific transplant regimens (SFTBI highest risk), diagnosis, and graft-versus-host disease (GVHD).
Conclusions:
- Pulmonary dysfunction is highly prevalent in long-term pediatric HCT survivors.
- Long-term follow-up and PFT are critical for early detection and management of lung issues.
- Specific transplant characteristics and GVHD influence the risk of pulmonary complications.
Background:
The purpose of this study was to determine the prevalence of pulmonary dysfunction in pediatric hematopoietic cell transplant (HCT) survivors and to identify associated risk factors.
Procedure:
In a cross-sectional study, patients surviving at least 5 years after pediatric HCT were requested to undergo pulmonary function testing (PFT). Risk factors for restrictive lung disease (RLD) and obstructive lung disease (OLD) were analyzed using multivariate analysis.
Results:
Among 472 patients contacted, 260 (55%) participated and 215 were selected for analysis. These patients were transplanted at a median age of 8.3 (0.3-18.0) years; 175 for hematologic malignancies and 40 for non-malignant diseases. The preparative regimens for 133 patients included fractionated TBI (FTBI), 29 single-fraction TBI (SFTBI), and 53 non-TBI regimens. PFT was performed at a median of 10 (5.0-27.5) years after HCT. Forty percent of patients had either RLD or OLD (28% RLD, 9% OLD, 3% mixed RLD/OLD) and at least 15% had an isolated low-DLCO. Moderate-to-severe impairment was present in 45% of patients with RLD or OLD. In multivariate analysis, risk factors associated with RLD included transplant regimen, transplant diagnosis, scleroderma/contracture, and donor relation. Patients treated with SFTBI had the highest risk of RLD. Risk factors for OLD included chronic graft-versus-host disease, transplant regimen, and time after HCT. Patients surviving 20 or more years after HCT had the highest risk of OLD.
Conclusions:
Fifty-five percent of long-term pediatric HCT survivors had pulmonary dysfunction. These findings stress the need for long-term follow-up to detect pulmonary dysfunction.