Abstract
Purpose :
Beta thalassemia major (B-TM) is an autosomal recessive disorder characterized by genetic mutations and resulting in the reduced synthesis of A globin chains. Another severe hemoglobinopathy is the sickle cell disease (SCD) which develops vasoocclusions with intravascular sickling, hemolysis, hemostasis and thrombosis. These patients require regular blood transfusions to survive. According to these transfusions and ischemic attacks, some ocular microvasculature abnormalities can be seen. We aimed to evaluate and compare the potential effect of these diseases to the choroid and retina with using spectral domain- optical coherence tomography (SD-OCT).
Methods :
This cross-sectional study included 22 β-thalassemia, 24 sickle cell disease patients and 24 healthy controls. Hemoglobin levels, serum ferritin levels and hematocrit levels of the B-TM and SCD patients were recorded. All of the participitants use oral chelators in our study. Right eyes of all participants underwent SD-OCT. We evaluated and compared choroidal thickness(CT), retinal nerve fiber layer (RNFL) thickness, central macular thickness (CMT) and ganglion cell layer (GCC) thickness of the participants. The normality of the values was analysed using using the Kolmogorov-Smirnov test. ANOVA test and post hoc analysis with the Tukey test were performed according to the Kolmogorov Smirnov test results.
Results :
Choroidal thickness was statistically higher in control group (353.79µm±71.93) than in B-TM (263.21 µm ±63.21) and SCD (306.36±46.02) group, at subfoveal region. In addition it was statistically higher in B-TM group than SCD group.(p=0.05) In SCD group, average RNFL was statistically significantly only thinner than control group, however there was no significant difference between B-TM and control or SCD group. In GCC layer was significantly thinner in SCD group than the other two groups. But there is no difference between B-TM and control group. The CMT values was not significantly different among three groups.
Conclusions :
These results show that ischemic effect of these diseases and/or side effects of the iron chelators are not limited to the periphery of the retina. In addition, we concluded that the ischemic effects of SCD in patients are more prominent than in those with B-TM, even if the proliferative or non-proliferative changes are not seen.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.