Thalassemia is a genetic in which the patient has a disorders of hemoglobin (Hb) formation. The distribution of the thalassemia gene is found around the Mediterranean Sea, the Middle East, India, along the lines of Southern China, Thailand, Malaysia, the Pacific islands, and Indonesia. Data from the Hematology-Oncology Working Group Unit of the Indonesian Pediatric Association (IDAI), as of April 2019, showed the number of thalassemia patients in Indonesia was 10,531 people.
In thalassemia-β major there is a disruption of the formation of globin-β chains, so that the globin-α chains become excessive. Excessive accumulation of globin-α chains in the red blood cell membrane causes damage to the membrane components including proteins and phospholipids. Furthermore, damages in red blood cells occur, also known as hemolysis, and the red blood cell formation system or erythropoiesis becomes ineffective. All of these mechanisms cause chronic anemia in thalassemia-β major patients.
The procedure that must be done is the provision of continuous blood transfusions. Hemolysis and repeated blood transfusions cause free iron levels in the body to increase. Free iron is a catalyst in the formation of free radicals that cause oxidative stress in the body. To protect cells from free radicals, there’s an endogenous antioxidant, one of them is glutathione that’s produce by the body. Glutathione consists of reduced glutathione and oxidized glutathione. Other antioxidant that can protect phospholipid membrane from oxidative stress is vitamin E (α-tocopherol). However, α-tocopherol cannot be produced in the body, or also known as an exogenous antioxidant.
Several previous studies have shown that α-tocopherol deficiency occurs in patients with thalassemia major, so it is recommended that routine supplementation of α-tocopherol be used as an antioxidant. But until now, there has been no guideline regarding routine α-tocopherol supplementation. Hematology-Oncology Work Coordination Unit IDAI provides α-tocopherol as one of the therapy of thalassemia major in Indonesia. However, the center of thalassemia in Children Hospital in Oakland and Thalassemia International Federation (TIF) didn’t recommend routine supplementation of α-tocopherol for thalassemia patient because α-tocopherol can be found in daily foods such as vegetable oil, nuts, and cereal.
Based on this background, research is needed to determine the role of α-tocopherol on hemolysis and oxidative stress in red blood cell membranes in thalassemia-β major patients who receive transfusion and routine iron chelation. A researcher from FMUI Biomedical Science Doctoral Program, dr. Nora Sovira, M.Ked(Ped), SpA(K), then conducted the research. The results of the study show that α-tocopherol can improve hemolysis in the red blood cell membrane of thalassemia-β major patients, as seen in the improvement of Hb levels after α-tocopherol administration, although Hb levels cannot reach norm values. This research also shows that oxidative stress can still happens in thalassemia-β major even if they already received routine transfusion and iron chelation.
The result was presented by dr. Nora Sovira, M.Ked(Ped), SpA(K) on her doctoral hearing on Wedesday June 19th 2019 in IMERI Auditorium. The dissertation is titled “Role of α-tocopherol on hemolysis markers and oxidative stress of Thalassemia-β major red blood cells”
Acted as the head of the examining team is Prof. Dr. dr. Suhendro, SpPD-KPTI with members of the examining team includes Prof. Dr. dr. Saptawati Bardosono, MSc; Prof. dr. Mohamad Sadikin, DSc; and Prof. dr. Munar Lubis, SpA (K) from North Sumatera University.
At the end of the hearing, Prof. Dr. dr. Rini Sekartini, SpA(K), as head of the hearing appointed Dr. Nora Sovira, M.Ked (Ped), SpA(K) as a Doctor in Medical Sciences of FMUI.
Through their closing remarks, promotor Dr. dr. Pustika Amalia Wahidiyat, SpA(K) and co-promotors Prof. dr. Fransiscus D. Suyatna, PhD, SpFK and Prof. dr. Djajadiman Gatot, SpA(K) hopes that the results of this study can be an input to the Hematology-Oncology Work Coordination Unit of IDAI in making new guidelines for thalassemia-β major treatment.
(Public Relations FMUI)