Back to Journals » Clinical Ophthalmology » Volume 13

Hemoglobin level and macular thinning in sickle cell disease

Authors Hussnain SA, Coady PA, Slade MD, Carbonella J, Pashankar F, Adelman RA, Stoessel KM

Received 19 November 2018

Accepted for publication 28 February 2019

Published 15 April 2019 Volume 2019:13 Pages 627—632


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Dr Scott Fraser

S Amal Hussnain,1–4 Patrick A Coady,1,5 Martin D Slade,6 Judith Carbonella,7 Farzana Pashankar,7 Ron A Adelman,1 Kathleen M Stoessel1

1Department of Ophthalmology and Visual Science, Yale University School of Medicine, New Haven, CT, USA; 2Department of Ophthalmology, Columbia University Vagelos College of Physicians and Surgeons, New York, NY, USA; 3Vitreous Retina Macula Consultants of New York, New York, NY, USA; 4Department of Ophthalmology, New York University School of Medicine, New York, NY, USA; 5New England Retina Associates, Hamden, CT, USA; 6Department of Internal Medicine, Yale University School of Medicine and Yale School of Public Health, New Haven, CT, USA; 7Department 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.

Keywords: sickle cell retinopathy, macular thinning, spectraldomain optical coherence tomography, hemoglobin

Creative Commons License This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at and incorporate the Creative Commons Attribution - Non Commercial (unported, v3.0) License. By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms.

Download Article [PDF]  View Full Text [HTML][Machine readable]