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Updated: Mar 29, 2026
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Lung function, transfusion, pulmonary capillary blood volume and sickle cell disease
Alan Lunt1, Emily McGhee2, Polly Robinson2
1Division of Asthma, Allergy and Lung Biology MCR Centre for Allergic Mechanisms in Asthma, King's College London, UK; National Institute for Health Research (NIHR) Biomedical Research Centre based at Guy's and St. Thomas' NHS Foundation Trust and King's College London, UK.
Insights
Blood transfusions in children with sickle cell disease (SCD) increase pulmonary capillary blood volume (PCBV) and respiratory system resistance (Rrs5), potentially explaining lung function abnormalities.
Area of Science:
- Pulmonary Medicine
- Pediatric Hematology
Background:
- Children with sickle cell disease (SCD) often exhibit lung function abnormalities.
- Elevated pulmonary blood volume is a potential contributing factor to these abnormalities.
Purpose of the Study:
- To investigate the acute effects of blood transfusion on pulmonary capillary blood volume (PCBV) and respiratory system resistance (Rrs5) in children with SCD.
- To determine if changes in PCBV correlate with changes in Rrs5.
Main Methods:
- 18 children with SCD underwent spirometry and Rrs5 measurements before and after blood transfusion.
- Pulmonary capillary blood volume (PCBV) was calculated using diffusing capacity for carbon monoxide and nitric oxide.
Main Results:
- Following transfusion, Rrs5 increased significantly (p<0.0001), as did PCBV (p<0.0001).
- Forced expiratory volume in one second and vital capacity decreased (p=0.0056 and p=0.0008, respectively).
- Increased Rrs5 correlated with increased PCBV (r=0.50, p=0.0493).
Conclusions:
- Acute blood transfusion in children with SCD leads to increased pulmonary capillary blood volume.
- This increase in PCBV may contribute to the observed lung function abnormalities, including increased respiratory system resistance.
Abstract:
Lung function abnormalities occur in children with sickle cell disease (SCD) and may be associated with elevated pulmonary blood volume. To investigate that association, we determined whether blood transfusion in SCD children acutely increased pulmonary capillary blood volume (PCBV) and increased respiratory system resistance (Rrs5). Measurements of Rrs5 and spirometry were made before and after blood transfusion in 18 children, median age 14.2 (6.6-18.5) years. Diffusing capacity for carbon monoxide and nitric oxide were assessed to calculate the PCBV. Post transfusion, the median Rrs5 had increased from 127.4 to 141.3% predicted (p<0.0001) and pulmonary capillary blood volume from 39.7 to 64.1 ml/m2 (p<0.0001); forced expiratory volume in one second (p=0.0056) and vital capacity (p=0.0008) decreased. The increase in Rrs5 correlated with the increase in PCBV (r=0.50, p=0.0493). Increased pulmonary capillary blood volume may at least partially explain the lung function abnormalities in SCD children.
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