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Pulmonary Function after Nonmyeloablative Hematopoietic Cell Transplant for Sickle Cell Disease
A Parker Ruhl1,2, Ruba Shalhoub3, Neal Jeffries3
1Laboratory of Malaria and Vector Research, National Institute of Allergy and Infectious Diseases.
Annals of the American Thoracic Society
|August 27, 2024
Summary
Hematopoietic cell transplant (HCT) stabilizes pulmonary function in adults with sickle cell disease (SCD). While forced expiratory volume in 1 second (FEV1) remained unchanged, diffusing capacity of the lung for carbon monoxide (DlCO) and 6-minute walk distance (6MWD) improved post-HCT.
Area of Science:
- Pulmonary Medicine
- Hematology
- Transplant Science
Background:
- Sickle cell disease (SCD) causes significant pulmonary complications due to vaso-occlusive events.
- Hematopoietic cell transplant (HCT) offers a potential cure for SCD, but its long-term effects on pulmonary function require further investigation, especially in adults.
- Understanding post-HCT pulmonary changes is crucial for guiding treatment decisions and managing patient care.
Purpose of the Study:
- To assess the stability of pulmonary function testing (PFT) parameters, including forced expiratory volume in 1 second (FEV1), three years after HCT in adults with SCD.
- To evaluate changes in diffusing capacity of the lung for carbon monoxide (DlCO) and 6-minute walk distance (6MWD) following HCT in this patient population.
Main Methods:
- A prospective cohort study of adults with SCD undergoing nonmyeloablative HCT was conducted.
- Pulmonary function tests (PFTs) and 6-minute walk distance (6MWD) were performed before HCT and annually for three years.
- Statistical analysis using generalized estimating equations was employed to assess changes in PFT parameters and 6MWD.
Main Results:
- Pulmonary function remained largely stable, with no significant changes in percent predicted FEV1 or forced vital capacity (FVC) three years post-HCT.
- A significant improvement was observed in diffusing capacity of the lung for carbon monoxide (DlCO), increasing by 3.7% (95% CI, 1.0% to 6.3%).
- The 6-minute walk distance (6MWD) also showed a significant increase of 25.9 meters (95% CI, 6.6 to 45.2 meters) three years after HCT.
Conclusions:
- Nonmyeloablative HCT appears to stabilize pulmonary function in adults with SCD and may halt ongoing vaso-occlusive pulmonary injury.
- Improvements in DlCO and 6MWD suggest potential benefits of HCT for cardiopulmonary health in SCD patients.
- Further multicenter studies are warranted to confirm these findings and evaluate long-term lung health outcomes in both adult and pediatric populations post-HCT for SCD.
