Everyone should have heard of thalassemia in life, but do you know about thalassemia? Today, the editor will let you know together what exactly causes thalassemia and what are the causes of thalassemia? Now, come and learn the relevant knowledge with us!

What causes thalassemia?

1. The causes of thalassemia are: continuous small amount of blood loss in the body, such as gastrointestinal tumors, hemorrhoids, ulcers, etc., which over time can cause anemia. It is the accelerated destruction of blood cells, such as hemolytic anemia. In addition to the above reasons, there is a special type of anemia, namely anemia caused by kidney disease, of which renal failure and uremia are the most common. Iron deficiency anemia is caused by a lack of iron, the raw material for making blood, resulting in reduced blood cell production. It is the function of the hematopoietic organ that malfunctions and cannot produce enough blood cells.

2. Thalassemia is caused by deletion or point mutation of the globin gene. There are four types of peptide chains that make up globin, namely,,,, and chains, which are encoded by their corresponding genes. Deletion or point mutation of these genes can cause synthesis obstacles of various peptide chains, resulting in changes in the components of hemoglobin.

3. Due to various mutations in the organization and structure of the globin gene, the gene expression occurs partially (a, less) or completely (a, p liver disorder), resulting in one or several normal diseases. A group of highly heterogeneous syndromes caused by reduced or absent globin chain synthesis. The clinical manifestations of different types of thalassemia vary greatly. The most severe cases may die before the fetus is born, while the mildest cases may remain anemic and asymptomatic throughout life. Most of the clinical findings are anemia patients between the two, with hypochromic small red blood cells and target-shaped red blood cells, and various changes in hemoglobin composition.

What are the dangers of thalassemia?

1. If the thalassemia is mild, it will not have much impact if it is well controlled. If it is moderate to severe, it will cause great pain to the patient, because people suffering from thalassemia When patients become ill, they will experience general weakness, malnutrition, hepatosplenomegaly, etc. As age increases, the condition will become more and more serious, and bronchitis and nephritis will also occur. Various organs will be damaged to a certain extent due to iron deposition, leading to heart failure and death.

2. Patients with thalassemia will put a huge burden on their families’ finances during the treatment of the disease. It is a hereditary disease itself, and it is very difficult to completely treat it. , but even in this way, it cannot be said that it can definitely be cured. It will cause great harm to the body and spirit of lung patients.

3. If a pregnant woman suffers from thalassemia, it will be even more serious. It will not only affect her own physical condition, but may also be transmitted to the fetus. Such children need lifelong blood transfusion treatment. It brings great pain to the patient and a lot of pressure to the family, so the only way to make adjustments is in the diet.

4. Mild ones will have a serious impact on the patient. If they are particularly serious, the harm will be great, causing great harm to people’s health, as well as to the patient’s body, psychology and spirit. All of them have a great impact, putting patients in long-term pain, eventually leading to malnutrition, which can be life-threatening.

How to treat thalassemia

1. General treatment: Pay attention to rest and nutrition, and actively prevent infection. Appropriate supplement of folic acid and vitamin E.

2. Blood transfusion and iron removal therapy: This method is still one of the important treatment methods at present.

The small amount of red blood cell transfusion method is only suitable for thalassemia intermedia and thalassemia, and is not recommended for thalassemia major. For thalassemia major, moderate and high-volume blood transfusions should be given from an early stage to bring the child's growth and development closer to normal and prevent bone lesions. The method is: first repeatedly infuse concentrated red blood cells to bring the hemoglobin content of the child to 120-150G/L; then infuse 10-15ML/KG of concentrated red blood cells every 2 to 4 weeks to maintain the hemoglobin content at 90-105G/L above. However, this method can easily lead to hemosiderinosis, so iron chelation treatment should be given at the same time.

3. Iron chelators: Deferoxamine is commonly used, which can increase the excretion of iron from urine and feces, but cannot prevent the absorption of iron by the gastrointestinal tract. Iron load assessment is usually performed one year after regular red blood cell transfusion or 10 to 20 units. If there is iron overload, such as SF1000G/L, iron chelating agents are started. Deferoxamine is 25 to 50 MG/KG per day, injected subcutaneously once a night for 12 hours, or added to isotonic glucose solution for intravenous infusion for 8 to 12 hours; 5 to 7 days a week for long-term use. Or add it to red blood cell suspension and infuse slowly. Deferoxamine has few side effects, but allergic reactions are occasionally seen. Long-term use of deferoxamine can cause cataracts and long bone development disorders. Excessive dosage can cause vision and hearing loss. The combined use of vitamin C and chelating agents can enhance the effect of deferoxamine on excreting iron from urine. The dose is 200RNG/day.

4. Splenectomy: Splenectomy has a better effect on H-blood disease and thalassemia intermedia, but has a poor effect on thalassemia major. Splenectomy can cause weakened immune function, so it should be performed after 5 to 6 years old and the indications should be strictly controlled.

5. Hematopoietic stem cell transplantation: Allogeneic hematopoietic stem cell transplantation is currently the method that can cure severe thalassemia. If an HLA-matched hematopoietic stem cell donor is available, it should be the first choice for treating thalassemia major.

6. Gene activation therapy: The application of chemical drugs can increase or decrease gene expression to improve the condition of thalassemia. Drugs that have been used clinically include urea and 5-azacytidine 5 ～AZC, cytarabine, malylan, isoniazid, etc. are currently being explored.