Unstable Hemoglobin Variants: Molecular Mechanisms, Clinical Phenotypes, and a Practical Diagnostic and Management Approach.

Abstract excerpt

Unstable hemoglobin variants are rare structural hemoglobin disorders in which alterations in the globin chain reduce hemoglobin stability, promoting denaturation, precipitation, and red cell injury. Affected individuals may present with a broad spectrum, ranging from compensated hemolysis to severe transfusion-dependent anemia, and episodes are commonly exacerbated by intercurrent illness or oxidative stress....

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