prev next front |1 |2 |3 |4 |5 |6 |7 |8 |9 |10 |11 |12 |13 |14 |15 |16 |17 |18 |19 |20 |21 |review
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.