Multiple transfused thalassemia major: ocular manifestations in a hospital-based population.
OBJECTIVE
To study the ocular manifestations in multiple transfused beta-thalassemia major patients and assess the ocular side-effects of iron chelating agents.


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.


CONCLUSIONS
Regular ocular examinations can aid in preventing, delaying or ameliorating the ocular complications of thalassemia.
Taneja
Rashi
R
Department of Ophthalmology, Din Dayal Upadhyay Hospital, Hari Nagar, New Delhi-110 064, India.
Malik
Pankaj
P
Sharma
Mamta
M
Agarwal
Mahesh C
MC
D000293:Adolescent; D001803:Blood Transfusion; D002386:Cataract / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D000077543:Deferiprone; D003676:Deferoxamine / Q000627:therapeutic use; D005128:Eye Diseases / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D007223:Infant; D007502:Iron Chelating Agents / Q000627:therapeutic use; D008297:Male; D011728:Pyridones / Q000627:therapeutic use; D055213:Retinal Pigment Epithelium / Q000473:pathology; D014792:Visual Acuity; D055815:Young Adult; D017086:beta-Thalassemia / Q000150:complications* / Q000628:therapy
D007502:Iron Chelating Agents; D011728:Pyridones; D000077543:Deferiprone; D003676:Deferoxamine