Thalassemia is a hemolytic anemia caused by partial or total inhibition of the synthesis of the peptide chains of the pearl protein in hemoglobin, resulting in ineffective production of red blood cells.
Indications for blood transfusion in thalassemia
1. Patients with intermediate alpha thalassemia (hemoglobin H disease) have a significant increase in anemia in the presence of infection or pregnancy, and in this case, if there are symptoms of compensatory anemia, then red blood cell transfusion is appropriate.
2. Once diagnosed, patients with beta-thalassemia major should be treated with blood transfusion as soon as possible. The reason for this is that these patients start to have anemia after 6 months of life, and thereafter the anemia is progressively aggravated. Patients have developmental disorders and gradually develop enlarged heart, enlarged liver and spleen, and bone lesions. Ineffective bone marrow hematopoiesis contributes to excessive iron absorption in the gastrointestinal tract and secondary hemochromatosis occurs. If blood transfusion is given as early as possible, especially “high volume” transfusion, the occurrence of these pathological changes can be delayed.
3. Patients with intermediate β-thalassemia also need temporary transfusion of red blood cells in case of infection, pregnancy, surgery and other stressful situations.
Blood transfusion protocol for thalassemia
1. “Medium volume” transfusion protocol
This regimen is an intermittent transfusion of red blood cells to maintain a “safe” Hb level of 60-70g/L. The purpose is to reduce the patient’s anemia. The purpose is to reduce the patient’s anemia symptoms so that life can be maintained. This transfusion is only a supportive treatment and cannot correct or alleviate the various pathological changes of the patient. This transfusion program is mostly used in China.
2.”High volume” transfusion program
This program is to maintain Hb at about 100g/L by transfusion of red blood cells. It is advisable to repeatedly transfuse for a short period of time at first, and then extend the interval of transfusion appropriately after the Hb reaches the above level. After this treatment, the patient’s hepatosplenomegaly and skeletal lesions are significantly reduced, and the absorption of iron in the gastrointestinal tract is also reduced.
3. “Ultra-high volume” transfusion protocol
This regimen is designed to maintain the patient’s Hb at about 130g/L. Patients treated with this transfusion regimen from infancy have been observed to have no typical thalassemia face and no pathological bone changes. Because this regimen greatly increases the need for blood transfusion and is not easily achieved, its use has been largely abandoned abroad.
Choice of blood products
1. Patients with thalassemia do not have the problem of insufficient blood volume, and those with transfusion indications can only transfuse red blood cells without whole blood transfusion.
2.The choice of red blood cell products should be decided according to the condition, and it is best to use red blood cells with white blood cells removed (e.g., red blood cells prepared by filtration or red blood cells obtained by single harvesting with a new type of blood cell separator).
3, the past has advocated the transfusion of young red blood cells to reduce the number of transfusions and extend the interval between transfusions, because it can only reduce the transfusion demand of 12 ﹪ to 16%, and young red blood cells are relatively cumbersome to prepare, high cost, now less used.
Risks of long-term blood transfusion
1. Excessive absorption of iron in the gastrointestinal tract of patients with thalassemia major, combined with long-term transfusion, can lead to hemochromatosis. This is because too much iron enters the patient’s body with blood transfusion, and the body’s ability to excrete iron is more constant, and the iron load overload involves organs or tissues, forming fibrotic lesions, affecting the function of organs or even causing death.
2. Long-term blood transfusion is prone to homoimmunity, which leads to adverse transfusion reactions and reduces the efficacy of transfusion, and can also spread diseases.
Therefore, long-term blood transfusion is not a long-term solution for patients with thalassemia. Patients urgently need to receive a treatment that can stabilize the disease and gradually get rid of blood transfusion, and it is recommended to combine Chinese and Western medicine to raise the value to a safe level as soon as possible.