disease ocular

2. Sickle cell disease is a group of chronic
hemolytic anemias, characterized by
vasculopathy, vaso-occlusive disease,
widespread acute and chronic organ damage,
and a reduced lifespan.
◼caused by a mutation in the β-globin gene

3. ◼Are heritable disorders in which the normal
adult hemoglobin is replaced by sickle
hemoglobin in red cell
◼ sickle-shaped deformity of the red cell on
deoxygenation
◼Cell passes through capillaries with difficulty
and is responsible for sluggish blood flow,
tissue hypoxia and infarction

4. 1. AS (Sickle cell trait)- mildest form; usually
requires severe hypoxia to produce sickling
2. SS (Sickle cell disease, sickle cell anemia)-
severe systemic complications such as
painful crisis and systemic hemolytic anemia
3. SC (Sickle C disease) & S Thal (Sickle cell
thalassemia)- associated with mild anemia
but severe ocular manifestations

5. ◼Conjunctival changes with comma shaped
capillaries
◼Proliferative sickle retinopathy: arterial
occlusions and peripheral capillary closure
which leads to new vessel formation ( sea fan
pattern)
◼Complications:
◼ Retinal detachment
◼ Vitreous hemorrhage