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With 3-gene
deletion alpha thalassemia, however, beta chains begin to associate in
groups of 4, producing an abnormal hemoglobin, called hemoglobin H. The
condition is sometimes called hemoglobin H disease. Hemoglobin H has 2
problems. First it does not carry oxygen properly, making it functionally
useless to the cell. Second, hemoglobin H protein damages the membrane that
surrounds the RBC, accelerating cell destruction. The combination of the
very low production of alpha chains and destruction of RBCs in hemoglobin H
disease produces a severe, life-threatening anemia. Untreated, most patients
die in childhood or early adolescence.
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