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beta thalassemia - Coggle Diagram
beta thalassemia
treatment
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spleen, gallbladder removal surgery
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clinical features
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b.t major
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enlarged spleen, liver and heart
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investigations
complete blood count, cbc
Hemoglobin electrophoresis with hemoglobin F and A2 quantitation: A lab test that differentiates the types of hemoglobin.
pathology
reduced b-globin synthesis, with relative excess of alpha-globin
inadequate HbA formation
hypochromic red cell > extravascular hemolysis/ destruction
of aggregate-containing red cells in spleen > anemia
accumulation of unpaired alpha-globin chains> toxic precipitates > damage the membrane of red cells and erythroid precursors > ineffective erythropoiesis > most erythroblasts die in bone marrow
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inappropriate absorption of dietary iron> systemic iron overload/secondary hemochromatosis in heart and liver