Introduction to the treatment of thalassemia

  Thalassemia is the most common incompletely dominant chronic hemolytic anemia in the population, also known as maritime anemia, and is a group of genetic disorders. The pathogenesis of the disease is the reduction or deletion of the hemoglobin chain that synthesizes hemoglobin, resulting in abnormal hemoglobin structure, reduced deformability of red blood cells containing abnormal hemoglobin, and shortened life span, which is also known as hemolytic anemia. In clinical practice, there are three types of thalassemia: mild, intermediate and severe. The mild and some of the intermediate types are usually survivable until adulthood, while the severe ones often die in early childhood due to chronic hemolysis or recurrent infections.  Thalassemia is mainly a preventive disease. If not, the patient will have to rely on blood transfusions, long-term iron removal, and the use of iron removal agents to maintain life. Even so, long-term blood transfusions will lead to the deposition of iron in the liver, spleen and other organs, which will cause the failure of these organs and lead to death. There is also a considerable risk of hematopoietic liver cell transplantation.  Pregnant women with thalassemia major should pay special attention to the fact that if the fetus is confirmed to have thalassemia major, it is better to terminate the pregnancy by abortion. If the test results show that the fetus is genetically normal or has a mild form of thalassemia, the pregnancy can be continued with peace of mind. Mild thalassemia does not require special treatment Intermediate and severe thalassemia can be treated with blood transfusions and iron removal, rest and nutrition, active prevention of infections, and appropriate folic acid and vitamin E supplementation.