Alpha-thalassemias Thalassemia

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the α-thalassemias involve genes hba1 , hba2, inherited in mendelian recessive fashion. 2 gene loci , 4 alleles exist. connected deletion of 16p chromosome. α thalassemias result in decreased alpha-globin production, therefore fewer alpha-globin chains produced, resulting in excess of β chains in adults , excess γ chains in newborns. excess β chains form unstable tetramers (called hemoglobin h or hbh of 4 beta chains), have abnormal oxygen dissociation curves.








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