Reduced red blood cell life

Heavy β thalassemia is a homozygote of β0 or β + thalassemia or a double heterozygote of β0 and β + thalassemia. Because β-chain generation is completely or almost completely inhibited, the synthesis of HbA containing β-chain is reduced or disappeared, and the excess The α chain is combined with the γ chain to become HbF (a2γ2), which significantly increases HbF. Due to the high oxygen affinity of HbF, the patient's tissue is hypoxic. Excess a-chains are deposited in young red blood cells and red blood cells, forming a-chain inclusions that attach to the erythrocyte membrane and make it stiff. Most of them are destroyed in the bone marrow and cause "ineffective hematopoiesis." Although some red blood cells containing inclusion bodies can mature and be released into peripheral blood, they are easily destroyed when they pass through the microcirculation; this type of inclusion body also affects the permeability of the red blood cell membrane, resulting in a shortened life span of red blood cells.

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