Risk factors for proliferative sickle retinopathy

Area of Science:

• Ophthalmology
• Hematology
• Genetics

Background:

• Sickle cell retinopathy, particularly proliferative sickle retinopathy (PSR), is a significant complication of sickle cell disease.
• Understanding the prevalence, incidence, and risk factors for PSR is crucial for patient management and preventative strategies.

Purpose of the Study:

• To investigate the prevalence, incidence, and risk factors of proliferative sickle retinopathy (PSR) in patients with homozygous sickle cell (SS) disease and sickle cell haemoglobin C (SC) disease.
• To identify demographic and hematological factors associated with PSR development in these patient groups.

Main Methods:

• Retrospective analysis of 786 patients with SS disease and 533 patients with SC disease.
• Assessment of PSR prevalence, incidence, and associated risk factors, including age, sex, and hematological parameters.

Main Results:

• PSR was more prevalent in SC disease (predominantly in males) and increased with age in both genotypes.
• Age-specific risk peaks for PSR were identified: 15-24 years (males) and 20-39 years (females) in SC disease; 25-39 years (both sexes) in SS disease.
• Hematological risk factors included high hemoglobin in males and low fetal hemoglobin in both sexes for SS disease, and high mean cell volume and low fetal hemoglobin in females for SC disease.

Conclusions:

• Proliferative sickle retinopathy (PSR) is more common in SC disease, with distinct age and sex risk profiles.
• Specific hematological parameters are associated with PSR risk in both SS and SC disease, highlighting potential targets for intervention.