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Competitive Transplants to Evaluate Hematopoietic Stem Cell Fitness
Published on: August 31, 2016
Effectiveness of long-term routine pulmonary function surveillance following pediatric hematopoietic stem cell
Dario Prais1, Moran Marx Sinik, Jerry Stein
1Pulmonary Institute, Schneider Children's Medical Center of Israel, Petach Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Insights
Long-term pulmonary function testing (PFT) surveillance after hematopoietic stem cell transplantation (HSCT) in children reveals mild restrictive lung disease in most survivors. Adherence to PFT protocols is crucial for early intervention in those at risk.
Area of Science:
- Pediatric Pulmonology
- Hematology-Oncology
- Stem Cell Transplantation
Background:
- Pulmonary complications are common after hematopoietic stem cell transplantation (HSCT), often remaining subclinical.
- Periodic pulmonary function testing (PFT) is essential for monitoring lung health in pediatric HSCT survivors.
Purpose of the Study:
- To evaluate the effectiveness of long-term PFT surveillance in children post-HSCT.
- To identify risk factors associated with pulmonary complications after HSCT.
Main Methods:
- Retrospective review of long-term PFT data from pediatric HSCT patients.
- Inclusion criteria: PFT performed before and at least once after HSCT.
Main Results:
- A gradual decrease in forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1) was observed, indicating a restrictive ventilatory defect.
- Allogeneic HSCT and radiation-based conditioning were associated with a greater decline in FVC and FEV1.
- Severe, irreversible airway obstruction developed in a small subset of patients (>2 years post-HSCT).
Conclusions:
- Most childhood HSCT survivors maintain near-normal lung function, but mild restrictive lung disease can develop.
- Allogeneic HSCT and radiation conditioning are risk factors for developing restrictive lung disease.
- Stringent adherence to PFT surveillance protocols is necessary for timely intervention to prevent irreversible pulmonary changes.
Introduction:
Pulmonary complications following hematopoietic stem cell transplantation (HSCT) are common and often subclinical. Thus, periodic pulmonary function testing (PFT) is mandatory. This study sought to evaluate the effectiveness of long-term PFT surveillance for children undergoing HSCT and identify potential risk factors.
Methods:
We reviewed long-term PFT for HSCT patients at a tertiary pediatric center. Inclusion criteria were PFT prior to and at least once following HSCT.
Results:
Fifty-seven patients performed 202 spirometry and 193 plethysmographic maneuvers; 41 were tested during the first year after HSCT, but only 29 were evaluated consistently long term (2-12 years). FVC and FEV(1) decreased gradually suggesting a restrictive ventilatory defect: FVC % predicted [mean ± SD] dropped from 91 ± 14% to 85 ± 17% after 0-24 months and 80 ± 19% beyond 2 years (P = 0.01) whereas FEV(1) dropped from 95 ± 16% to 88 ± 19% and 82 ± 20%, respectively (P = 0.002). A slight reduction in TLC was observed. Those undergoing allogeneic HSCT had a greater decline in FVC (P = 0.025) and FEV(1) (P = 0.025) as did those conditioned with radiation, regarding both FVC (P = 0.003) and FEV(1) (P = 0.002). Decline occurred earlier (≤2 years) after chemotherapy compared with radiation. Seven children had severe irreversible obstruction at >2 years despite therapeutic intervention.
Conclusions:
Most survivors of childhood HSCT maintain almost normal pulmonary function although mild restrictive lung disease may develop, particularly following allogeneic HSCT and conditioning with radiation. Severe airways obstruction developed in a small minority. The surveillance protocol for PFT needs to be followed more stringently to enable intervention possibly before early subclinical changes progress and become irreversible.

