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sickle-thalassemia
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sickle-thalassemia

pathology
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Quick Summary

Sickle-thalassemia is a complex blood disorder that arises from inheriting genetic defects affecting hemoglobin production. Hemoglobin is the protein in red blood cells responsible for oxygen transport. In thalassemia, there's a reduced rate of synthesizing globin chains, leading to insufficient hemoglobin and abnormally shaped, small red blood cells that are destroyed prematurely.12

Sickle cell anemia, another inherited condition, involves a variant hemoglobin (HbS) that causes red blood cells to deform into a rigid sickle shape when oxygen levels are low. These sickled cells can block blood vessels, causing pain and tissue damage.3

Sickle-thalassemia occurs when an individual inherits genes for both sickle cell anemia and thalassemia. This combination results in a more severe form of anemia than either condition alone, characterized by the production of both abnormal sickle-shaped cells and deficient hemoglobin.1

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3 Britannica Sources
  1. blood disease - Thalassemia and hemoglobinopathies
  2. thalassemia
  3. sickle cell anemia

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