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Clin Ophthalmol. 2019 Apr 15;13:627-632. doi: 10.2147/OPTH.S195168. eCollection 2019.

Hemoglobin level and macular thinning in sickle cell disease.

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Department of Ophthalmology and Visual Science, Yale University School of Medicine, New Haven, CT, USA.
Department of Ophthalmology, Columbia University Vagelos College of Physicians and Surgeons, New York, NY, USA.
Vitreous Retina Macula Consultants of New York, New York, NY, USA.
Department of Ophthalmology, New York University School of Medicine, New York, NY, USA.
New England Retina Associates, Hamden, CT, USA.
Department of Internal Medicine, Yale University School of Medicine and Yale School of Public Health, New Haven, CT, USA.
Department of Pediatric Hematology and Oncology, Yale University School of Medicine, New Haven, CT, USA.


Purpose: To study the relationship between complete blood count (CBC) indices over time, particularly serum hemoglobin (Hb) levels, and severity of macular thinning on spectral domain optical coherence tomography (SD-OCT) in patients with sickle cell disease (SCD). Methods: This is a single-center, retrospective analysis of 141 consecutive SCD patients over a 10-year period, of which 40 patients (79 eyes) had SD-OCT imaging of the macula and 29 (58 eyes, mean age 17.5 years) were eligible for the study. Investigators reviewed electronic medical records for documentation of retinopathy stage, disease genotype, CBC values, and SD-OCT imaging. SD-OCT parameters and CBC values were compared between different retinopathy stages and disease genotypes. Regression analyses were performed on SD-OCT parameters and CBC values. Results: Of the 58 eligible eyes (34HbSS, 18HbSC, 4HbSβ +thal, 2HbS βthal), 18 had PSR (proliferative sickle retinopathy), 14 had NPSR (nonproliferative sickle retinopathy), and 26 had NSR (no sickle retinopathy). Hb values were higher in SC group compared to SS group. Macular thickness in the temporal inner (Δ=26±33 um, p=0.01) and outer (Δ=21±30 um, p=0.02) subfields was higher in SC compared to SS group. Patients with SD-OCT thinning below the 5th percentile in the temporal outer subfields had lower recorded Hb nadirs (6.0±0.9) compared to those with thickness within the top 95th percentile (9.1±2.3). Regression analysis showed temporal macular thickness to be positively correlated with Hb values in the SS group. Conclusion: Macular thinning observed on SD-OCT in SCD patients with SS genotype may be related to the level of anemia in this population.


hemoglobin; macular thinning; sickle cell retinopathy; spectraldomain optical coherence tomography

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