β-Thalassemia and ocular implications: a systematic review
Aliki Liaska1, Petros Petrou1, Constantinos D Georgakopoulos1
11st Department of Ophthalmology, Athens University, Mesogeion 154, Athens, 11527, Greece.
BMC Ophthalmology
|July 9, 2016
Summary
Beta-thalassemia patients frequently experience ocular issues, including vision-impairing fundus changes similar to pseudoxanthoma elasticum. Regular eye exams are crucial for early detection and management of these beta-thalassemia complications.
Area of Science:
- Ophthalmology
- Hematology
- Genetics
Background:
- Beta-thalassemia is a severe inherited blood disorder requiring lifelong transfusions.
- Ocular complications are common in beta-thalassemia patients and can be serious.
Purpose of the Study:
- To determine the prevalence and spectrum of ocular abnormalities in beta-thalassemia.
- To identify risk factors associated with ocular changes in beta-thalassemia.
Main Methods:
- Review of observational studies on beta-thalassemia patients.
- Assessment of ocular abnormalities through clinical examination and multimodal imaging.
Main Results:
- Ocular involvement frequency ranged from 41.3-85%.
- Common findings include ocular surface disease, lens opacities, and pseudoxanthoma elasticum (PXE)-like fundus changes.
- PXE-like changes correlated with age, splenectomy, and disease duration; retinal vascular tortuosity was linked to anemia severity.
Conclusions:
- Beta-thalassemia presents with diverse structural and functional ocular signs.
- PXE-like fundus changes are frequent and increase with disease severity.
- Regular ophthalmic checkups are vital for patients with long-standing beta-thalassemia to monitor for PXE-like changes and potential choroidal neovascularization.
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