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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
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Sickle cell retinopathy. A focused review
Maram E A Abdalla Elsayed1, Marco Mura2, Hassan Al Dhibi2
1Jeddah Eye Hospital, Jeddah, Kingdom of Saudi Arabia.
Summary
Sickle cell retinopathy arises from abnormal hemoglobin, causing vision-threatening complications. Advances in imaging and potential therapies like gene therapy offer hope, but optimal management requires further study.
Area of Science:
- Ophthalmology
- Hematology
- Genetics
Background:
- Sickle cell retinopathy stems from abnormal hemoglobin aggregation in retinal microcirculation.
- This leads to red blood cell deformability issues, stagnant blood flow, thrombosis, and ischemia, potentially triggered by hypoxia, acidosis, or hyperosmolarity.
Purpose of the Study:
- To review recent advances in sickle cell retinopathy, including pathogenesis, imaging, management, and increasing prevalence.
- To highlight the condition's impact and potential future directions in the Western world.
Main Methods:
- A non-systematic, focused literature review was conducted.
Main Results:
- Sickle retinopathy can cause severe complications like vitreous hemorrhage and retinal detachment.
- New imaging techniques (wide-field, OCT angiography) improve early diagnosis.
- ANGPTL-4 is a potential mediator for proliferative retinopathy; laser therapy and surgical interventions are discussed.
- Gene therapy and stem cell transplantation are emerging as potentially curative treatments.
Conclusions:
- Optimal management strategies for sickle cell retinopathy lack strong evidence.
- Further research is needed to translate current understanding and diagnostic progress into improved patient outcomes.
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