法医学ジャーナル

The Interplay among Immune Response, Inflammation, Oxidative Stress and Sickle Cell Anaemia Pathogenesis

Abstract

Oduola Oguntibeju

Sickle Cell Anemia (SCD) stands as a life-threatening hematological condition primarily prevalent in sub-Saharan Africa. It originates from a genetic mutation in the β-chain hemoglobin gene, leading to the substitution of valine for glutamic acid. This genetic alteration gives rise to an aberrant hemoglobin variant known as hemoglobin S (HbS). Under deoxygenated conditions, HbS undergoes polymerization, causing red blood cells to assume a rigid, sickle-like shape and significantly reducing their lifespan. Scientific investigations have underscored a robust correlation between oxidative stress, inflammation, immune response, and the development of sickle cell disease. The cumulative effects of these processes contribute to the emergence of vasculopathy, affecting blood vessels, and a range of other complications. While recent research has elucidated the pivotal role of the innate immune system in SCD pathogenesis, insights into the involvement of the adaptive immune system remain limited. This comprehensive review delves into the intricate interplay among the immune system, inflammation, oxidative stress, blood transfusion, and their collective impact on the progression of sickle cell anemia.