Abstract
This review outlines the current clinical research investigating how the haptoglobin (Hp) genetic polymorphism and stroke occurrence are implicated in sickle cell disease (SCD) pathophysiology. Hp is a blood serum glycoprotein responsible for binding and removing toxic free hemoglobin from the vasculature. The role of Hp in patients with SCD is critical in combating blood toxicity, inflammation, oxidative stress, and even stroke. Ischemic stroke occurs when a blocked vessel decreases oxygen delivery in the blood to cerebral tissue and is commonly associated with SCD. Due to the malformed red blood cells of sickle hemoglobin S, blockage of blood flow is much more prevalent in patients with SCD. This review is the first to evaluate the role of the Hp polymorphism in the incidence of stroke in patients with SCD. Overall, the data compiled in this review suggest that further studies should be conducted to reveal and evaluate potential clinical advancements for gene therapy and Hp infusions.
Highlights
From the literature we reviewed, we observed that most clinical outcomes were insignificantly correlated with the Hp genotype
This review was conducted to elucidate a connection between sickle cell disease (SCD), Hp, and stroke
To make any applicable conclusions on this topic, more clinical studies should be conducted with an emphasis on finding correlations between various Hp genotypes and relevant SCD outcomes
Summary
Sickle cell disease (SCD) is a group of autosomal recessive disorders that affect an estimated 20 to 25 million people worldwide [1], making it the most prevalent monogenic disorder and a serious public health concern. Inheritance of this disorder is concentrated in sub-Saharan African, South Asian, Middle Eastern, and Mediterranean regions [2,3]. SCD is a hemolytic disorder caused by a range of mutations in the gene responsible for coding the β-globin (HBB) subunits of hemoglobin (Hb) These mutations usually result in abnormal versions of Hb, which are capable of polymerizing, leading to malformed, sickle-shaped red blood cells (RBCs). A direct product of Hb destruction, is the main culprit of many symptoms associated with SCD
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