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Multiple transfused thalassemia major: ocular manifestations in a hospital-based population
Rashi Taneja1, Pankaj Malik, Mamta Sharma
1Department of Ophthalmology, Din Dayal Upadhyay Hospital, Hari Nagar, New Delhi-110 064, India.
Insights
Ocular complications are common in beta-thalassemia major patients receiving multiple blood transfusions. Regular eye exams are crucial for managing these vision issues and potential side effects from iron chelation therapy.
Area of Science:
- Ophthalmology
- Hematology
- Pediatrics
Background:
- Beta-thalassemia major requires frequent blood transfusions, leading to iron overload.
- Iron overload can cause significant systemic and ocular complications.
- Iron chelation therapy is essential but may have its own side effects.
Purpose of the Study:
- To investigate ocular manifestations in transfusion-dependent beta-thalassemia major patients.
- To evaluate ocular side effects associated with iron chelating agents.
- To establish the role of regular ophthalmic examinations in managing thalassemia-related eye conditions.
Main Methods:
- Prospective observational study of 45 beta-thalassemia major children (6 months-21 years).
- Patients grouped by transfusion and iron chelation regimens (desferrioxamine, deferiprone).
- Ocular status assessed at enrollment and quarterly for one year.
Main Results:
- 58% of patients exhibited ocular involvement, most commonly lenticular opacities (44%) and decreased visual acuity (33%).
- Ocular changes correlated with higher serum ferritin/iron levels and increased transfusion frequency.
- Desferrioxamine showed a protective effect on RPE mottling; both chelators correlated with specific ocular findings.
Conclusions:
- Regular ocular examinations are vital for early detection and management of ocular complications in thalassemia.
- Ophthalmic monitoring can help prevent, delay, or mitigate vision impairment.
- Understanding chelation therapy's ocular effects aids in optimizing patient care.
Purpose:
To study the ocular manifestations in multiple transfused beta-thalassemia major patients and assess the ocular side-effects of iron chelating agents.
Materials And Methods:
In this prospective observational study, 45 multiple transfused beta-thalassemia major children between six months and 21 years of age were enrolled and assigned groups according to the treatment regimens suggested. Group A received only blood transfusions, Group B blood transfusions with subcutaneous desferrioxamine, Group C blood transfusions with desferrioxamine and oral deferriprone and Group D blood transfusions with deferriprone. Ocular status at the time of enrolment was documented. Subjects were observed quarterly for one year for changes in ocular status arising due to the disease process and due to iron chelation therapy. Children with hemoglobinopathies other than beta-thalassemia major, congenital ocular anomalies and anemia due to other causes were excluded.
Results:
Ocular involvement was observed in 58% of patients. Lenticular opacities were the most common ocular finding (44%), followed by decreased visual acuity (33%). An increased occurrence of ocular changes was observed with increase of serum ferritin and serum iron levels as well as with higher number of blood transfusions received. Desferrioxamine seemed to have a protective influence on retinal pigment epithelium (RPE) mottling. Occurrence of lenticular opacities and RPE degeneration correlated positively with use of desferrioxamine and deferriprone respectively. Follow-up of patients for one year did not reveal any change in ocular status.
Conclusion:
Regular ocular examinations can aid in preventing, delaying or ameliorating the ocular complications of thalassemia.
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