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Conjunctival vessel abnormalities in sickle cell diseases: the influence of age and genotype
W C Siqueira1, M S Figueiredo, A A Cruz
1Department of Ophthalmology, School of Medicine, Ribeirão Preto, Brazil.
Insights
Sickle cell disease patients show similar conjunctival vessel abnormalities. These vascular changes worsen with age and are more severe in sickle cell anaemia patients compared to other sickle cell disease genotypes.
Area of Science:
- Ophthalmology
- Hematology
- Genetics
Background:
- Sickle cell diseases (SCDs) encompass a group of inherited blood disorders.
- Vascular complications are a hallmark of SCDs, affecting various organ systems.
- Conjunctival microvasculature can serve as a window to systemic vascular changes in SCDs.
Purpose of the Study:
- To investigate and grade conjunctival vessel abnormalities in patients with different sickle cell disease genotypes.
- To compare the severity of these abnormalities across sickle cell anaemia, sickle cell beta (0)-thalassaemia, and HbS/HbC disease.
- To assess the relationship between patient age and the severity of conjunctival vascular abnormalities in sickle cell disease.
Main Methods:
- Slit-lamp examination of conjunctival vessels was performed.
- Abnormalities were graded in 77 patients across three SCD genotypes.
- Data analysis focused on genotype-specific severity and age-related trends.
Main Results:
- Similar conjunctival vessel abnormalities were observed in all three SCD genotypes studied.
- Vascular abnormalities were more severe in HbS homozygotes (sickle cell anaemia) than in sickle cell beta (0)-thalassaemia and HbS/HbC disease.
- A positive correlation was found between patient age and the severity of vascular abnormalities in HbS homozygotes.
Conclusions:
- Conjunctival vascular abnormalities are a common feature across major sickle cell disease genotypes.
- The severity of these ocular vascular changes increases with age, particularly in sickle cell anaemia.
- Conjunctival lesions appear to accumulate and worsen similarly to other tissue damage in sickle cell diseases, highlighting potential for monitoring disease progression.
Abstract:
Conjunctival vessels were examined with a slit-lamp and the abnormalities were graded in 77 patients with sickle cell diseases, including 48 with sickle cell anaemia, 17 with sickle cell beta (0)-thalassaemia and 12 with HbS/HbC disease. The study demonstrated the occurrence of similar conjunctival vessel abnormalities for the three sickle cell diseases which are, however, more severe in HbS homozygotes than in the two other genotypes. An increase of the severity of the vascular abnormalities with the increase of patient age was also observed for HbS homozygotes. The data suggest that the conjunctival lesions accumulate and increase in severity in a similar fashion as other tissue damages observed in sickle cell diseases.