Ocular Manifestations of Sickle Cell Disease in Different Genotypes

Sickle cell disease (SCD) is a multisystemic disorder with variable systemic involvement which varies according to genotype. In this study, our aim is to compare ocular complications between HbSS, HbSC, HbS/β+ thalassemia, HbS/β0 thalassemia, SS alpha thalassemia, and S/β0 + alpha thalassemia genoty...

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Bibliographic Details
Published in:Ophthalmic epidemiology 2021-05, Vol.28 (3), p.185-190
Main Authors: AlRyalat, Saif Aldeen, Jaber, Bahaa Al-Din Mustafa, Alzarea, Abdulaziz A., Alzarea, Abdullah A., alosaimi, Wejdan A., Al Saad, Mouna
Format: Article
Language:English
Subjects:
eye
genotype
ocular
retinopathy
Sickle cell
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Summary:Sickle cell disease (SCD) is a multisystemic disorder with variable systemic involvement which varies according to genotype. In this study, our aim is to compare ocular complications between HbSS, HbSC, HbS/β+ thalassemia, HbS/β0 thalassemia, SS alpha thalassemia, and S/β0 + alpha thalassemia genotypes. Data of patients included in this study was recruited from the Cooperative Study of Sickle Cell Disease (CSSCD). Patients with major sickle cell hemoglobinopathies (SS, SC, Sβ- thalassemia, SS alpha thalassemia) were eligible for enrollment, after that, a detailed eye exam was performed. We categorized ocular complications into conjunctival sign, iris atrophy, and both proliferative and non-proliferative sickle cell retinopathy. A total of 1867 patients were included in this study, with a mean age of 27.7 (± 11.7) years. They were 830 (44.5%) males and 1037 (55.5%) females. The most common genotype was SS with 971 (52%) patients, and the least common form was sickle cell with both alpha and beta thalassemia major with 42 (2.2%) patients. We found a significant difference in the frequency of proliferative sickle cell retinopathy, where SC genotype had the highest frequency and S B0 thalassemia genotype had the lowest frequency. We also found a significant difference in the frequency of conjunctival sign, where SS genotype had the highest frequency and the S B+ thalassemia has the lowest frequency. We identified ocular complications for major sickle cell hemoglobinopathies, where we confirmed previous small study's findings and identified ocular complications of less common hemoglobinopathies.
ISSN:0928-6586
1744-5086
DOI:10.1080/09286586.2020.1801762