In beta-thalassemia, which globin will form unstable aggregates that result in RBC destruction?

In beta-thalassemia, which globin will form unstable aggregates that result in RBC destruction?

• A. B globin
• B. G globin
• C. D globin
• D. A globin

Answer: (C)

In beta-thalassemia the problem is a shortage of beta-globin, which leaves alpha-globin chains in excess. These unpaired alpha chains are unstable and precipitate within developing erythroid cells and in circulating red cells, forming insoluble aggregates that damage the cell membrane and cytoskeleton. This leads to red cell destruction, both through ineffective erythropoiesis in the marrow and hemolysis in the spleen. So the globin forming the unstable aggregates in this scenario is the alpha-globin chain.

In beta-thalassemia the problem is a shortage of beta-globin, which leaves alpha-globin chains in excess. These unpaired alpha chains are unstable and precipitate within developing erythroid cells and in circulating red cells, forming insoluble aggregates that damage the cell membrane and cytoskeleton. This leads to red cell destruction, both through ineffective erythropoiesis in the marrow and hemolysis in the spleen. So the globin forming the unstable aggregates in this scenario is the alpha-globin chain.