In beta thalassemia, there is –
Correct Answer: Decrease in beta chain, increase in alpha chain
Description: Beta - thalassemia
The abnormality common to all P-thalassemias is diminished synthesis of structurally normal β-globin chains, coupled with unimpaired synthesis of α-chain.
The reduced supply of (β-globin chain diminishes production of hemoglobin tetramers, causing hypochromic and microcytic anemia.
The haematological consequences of diminished synthesis of one globin chain stem not only from low intracellular hemoglobin, but also from a relative excess of the unimpaired chain.
Consequences of thalassemia
In (β-thalassemia, anemia is produced by two mechanisms
Deficit in HbA synthesis produce hypochromic microcytic anemia.
Excess free α-chains aggregate into insoluble inclusions within red cells and their precursors (normoblasts). These inclusions cause membrane damage that results in apoptosis of normoblasts in the marrow → ineffective erythropoiesis. The red cells which escape death in bone marrow are prone to splenic sequestration and destruction due to cell membrane damage and decreased deformability, leading to extravascular hemolysis.
The second mechanism is more important in producing anemia.
Anaemia leads to several additional problems -
Increased erythropoietin secretion in response to anemia leads to massive erythroid hyperplasia in bone marrow that results in expansion of bone marrow and skeletal deformities.
Erythropoietin also induces extramedullary erythropoiesis in liver and spleen -p Hepatosplenomegaly.
Excessive absorption of iron (in response to ineffective erythropoiesis) along with repeated blood transfusion in severe β-thalassemia results in iron overload and secondary hemochromatosis → iron overload in heart, liver, endocrine glands, spleen -4 cardiac failure / endocrine deficiency → Death.
Category:
Pediatrics
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