Difficult Cases in the Diagnosis of Thalassemia Syndromes.

Abstract excerpt

Thalassemia syndromes result from a significant reduction in the synthesis of globin chains (α, β, γ, δ). An imbalance between α- and non-α-globin chains underlies β-thalassemia. α-Globin tetramers accumulate and precipitate in erythroid precursors (ineffective erythropoiesis). Hemolysis also plays an important role, shortening the erythrocyte lifespan. α-Thalassemia syndromes are caused by approximately 200...

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