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Proliferative sickle retinopathy is the most severe ocular complication of sickle cell disease. Even though PSCR begins in the first decade of life, the condition remains asymptomatic and unnoticed until visual symptoms occur due to vitreous hemorrhage or retinal detachment.
Hyperviscosity occurs from pathologic changes of either cellular or protein fractions of the blood such as is found in polycythemias, multiple myeloma (particularly IgA and IgG3), leukemia, monoclonal gammopathies such as Waldenström macroglobulinemia, sickle cell anemia, and sepsis. [citation needed]
Sickle cell disease (SCD), also simply called sickle cell, is a group of hemoglobin-related blood disorders that are typically inherited. [2] The most common type is known as sickle cell anemia. [2] Sickle cell anemia results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. [2]
Retinopathy: If left untreated, any type of retinopathy (including diabetic retinopathy, hypertensive retinopathy, sickle cell retinopathy, anemic retinopathy, [8] etc.) can damage retina and lead to visual field defects and blindness. [9] Hypervitaminosis A: Excess consumption of vitamin A can cause blurred vision. [10]
Treatment is based on the cause of the retinopathy and may include laser therapy to the retina. Laser photocoagulation therapy has been the standard treatment for many types of retinopathy. Evidence shows that laser therapy is generally safe and improves visual symptoms in sickle cell and diabetic retinopathy.
Diabetes, for example, is the leading cause of new cases of blindness in those aged 20–74, with ocular manifestations such as diabetic retinopathy and macular edema affecting up to 80% of those who have had the disease for 15 years or more.
It also occurs in diabetes mellitus and is one of the causative factors for microvascular occlusion in diabetic retinopathy. So, the presence of rouleaux can also be a cause of disease because it will restrict the flow of blood throughout the body because capillaries can only accept free-flowing singular and independent red blood cells.
The sickle cell trait provides a survival advantage against malaria fatality over people with normal hemoglobin in regions where malaria is endemic. The trait is known to cause significantly fewer deaths due to malaria, especially when Plasmodium falciparum is the causative organism.
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