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Updated: Aug 6, 2025
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A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
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Hemoglobin and End-Organ Damage in Individuals with Sickle Cell Disease
William B Ershler1, Laura M De Castro2, Zahra Pakbaz3
1Department of Hematology, Inova Schar Cancer Institute, Fairfax, Virginia.
Current Therapeutic Research, Clinical and Experimental
|March 23, 2023
Summary
Higher hemoglobin levels in sickle cell disease (SCD) patients correlate with a lower risk of end-organ damage (EOD). Maintaining elevated Hb levels may improve patient outcomes and reduce healthcare costs.
Area of Science:
- Hematology
- Vascular Medicine
- Chronic Disease Management
Background:
- Sickle cell disease (SCD) is a complex inherited blood disorder.
- Anemia is a common complication of SCD, linked to end-organ damage (EOD).
Purpose of the Study:
- To investigate the association between hemoglobin (Hb) levels and the risk of EOD in adolescent and adult SCD patients.
- To systematically characterize the relationship between Hb and EOD in a large patient cohort.
Main Methods:
- Retrospective, observational, repeated-measures study design.
- Analysis of Hb data and EOD onset (kidney disease, pulmonary hypertension, stroke, leg ulcers) in SCD patients (≥12 years).
- Multivariable generalized estimating equations used to assess Hb level and EOD risk.
Main Results:
- 16,043 unique patients and 44,913 observations were analyzed.
- Higher Hb levels were significantly associated with lower odds of developing any EOD within one year.
- Comparable risk reductions for chronic kidney disease, pulmonary hypertension, and leg ulcers were observed with higher Hb levels.
Conclusions:
- Elevated Hb levels are linked to a reduced risk of EOD in individuals with SCD.
- Therapeutic approaches aimed at increasing Hb levels may provide significant clinical and economic benefits for SCD patients.
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