Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Observational Study
. 2025 Oct;72(10):e31951.
doi: 10.1002/pbc.31951. Epub 2025 Aug 1.

The Protective Effect of Fetal Hemoglobin on Retinal Damage in Sickle Cell Disease Patients: A Correlation With Optical Coherence Tomography, Hematologic Parameters, and Hydroxyurea Treatment

Jing Jin  1 , Pimpiroon Ploysangam  1 , Dorothy H Hendricks  1 , Robin E Miller  2
Affiliations
Observational Study

The Protective Effect of Fetal Hemoglobin on Retinal Damage in Sickle Cell Disease Patients: A Correlation With Optical Coherence Tomography, Hematologic Parameters, and Hydroxyurea Treatment

Jing Jin et al. Pediatr Blood Cancer. 2025 Oct.

Abstract

Purpose: The protective effect of fetal hemoglobin (HbF) on retinal health in patients with sickle cell disease (SCD) has been well recognized. Hydroxyurea, a disease-modifying agent, induces HbF production and modifies hematologic parameters. This study investigated the correlation between retinal damage on optical coherence tomography (OCT) and HbF levels. Additionally, it compared retinal OCT, HbF, and other hematologic laboratory parameters in patients treated with/without hydroxyurea.

Methods: In this prospective, observational study, 138 patients with SCD underwent ophthalmologic examination, including OCT. Hematologic values and treatment history were obtained from medical records. Analyses focused on correlations between HbF levels and hematologic markers, HbF trends with age in children with/without abnormal retinal OCT, and the impact of hydroxyurea treatment on HbF levels and OCT results.

Results: Children with normal retinal OCT exhibited a slower age-related decline in HbF levels before age 15 years compared with those with abnormal OCT. HbF levels showed a strongly negative correlation with markers of hemolysis and inflammation and a positive correlation with hemoglobin. Hydroxyurea treatment was associated with elevated HbF levels and a shift in hematologic profile toward those observed in patients with normal OCT findings. Children treated with hydroxyurea exhibited slower progression of macular thinning with age.

Conclusions: Children with normal OCT findings maintained higher HbF levels and showed a slower decline with age. Hydroxyurea treatment increases HbF levels, improves hematologic profiles, and reduces the likelihood of retinal abnormalities. Further research is needed to elucidate the mechanisms behind these observations and validate the findings in larger datasets.

Keywords: OCT; fetal hemoglobin; hydroxyurea; sickle cell retinopathy.

PubMed Disclaimer

References

    1. C. C. Chow, M. A. Genead, A. Anastasakis, F. Y. Chau, G. A. Fishman, and J. I. Lim, “Structural and Functional Correlation in Sickle Cell Retinopathy Using Spectral‐Domain Optical Coherence Tomography and Scanning Laser Ophthalmoscope Microperimetry,” American Journal of Ophthalmology 152, no. 4 (2011): 704–711.e2, https://doi.org/10.1016/j.ajo.2011.03.035.
    1. M. T. B. C. Bonanomi and M. M. Lavezzo, “Sickle Cell Retinopathy: Diagnosis and Treatment,” Arquivos Brasileiros De Oftalmologia 76, no. 5 (2013): 320–327, https://doi.org/10.1590/s0004‐27492013000500016.
    1. J. Jin, R. Miller, J. Salvin, et al., “Funduscopic Examination and SD‐OCT in Detecting Sickle Cell Retinopathy Among Pediatric Patients,” Journal of the American Association for Pediatric Ophthalmology and Strabismus 22, no. 3 (2018): 197–201.e1, https://doi.org/10.1016/j.jaapos.2017.12.019.
    1. J. Jin, V. Kandula, and R. E. Miller, “Monitoring Retinal Pathology and Cerebral Injury in Sickle Cell Disease Using Spectral‐domain Optical Coherence Tomography in Pediatric Patients,” Pediatric Blood & Cancer 68, no. 7 (2021): e29028, https://doi.org/10.1002/pbc.29028.
    1. H. Bard, “The Postnatal Decline of Hemoglobin F Synthesis in Normal Full‐Term Infants,” Journal of Clinical Investigation 55, no. 2 (1975): 395–398, https://doi.org/10.1172/JCI107943.

Publication types

MeSH terms

LinkOut - more resources