β-THALASSEMIA AND DISEASE TOLERANCE
Keywords:
ferritin, splenectomy, hemosiderosis, immunoglobulins, interleukins.Abstract
Susceptibility to infections made it necessary to study the immune status of patients with β-thalassemia. This study examined the role of iron overload syndrome (hemosiderosis) and the interaction of pathophysiological determinants of thalassemia and components of the immune system. Chronic antigenic stimulation and oxidative stress from iron overload are two major pathophysiological factors in thalassemia that affect the immune system. Life-long blood transfusion therapy for thalassemia with its uncontrolled negative effect on the immune and coagulation systems of the blood leads to the accumulation of iron in the tissues and immunosuppression and, as a consequence, to the addition of intercurrent infection and immunization. The role of immunological interactions in the pathogenesis of β-thalassemia needs further investigation in future studies.
References
REFERENCES:
1. Ali & Shahramian, Iraj & Yaghoobi, Hajar & Naderi, Majid & Azizi, Hakim. (2017). The Role of Immune System in Thalassemia Major: A Narrative Review. Journal of Pediatrics Review. In Press. 10.5812/jpr.14508.
2. Azarkeivan A et. al, Correlation between Heart, Liver and Pancreas Hemosiderosis Measured by MRI T2* among Thalassemia Major Patients from Iran. Arch Iran Med. 2016 Feb;19(2):96-100. doi: 0161902/AIM.006.
3. Kountouras D, et al, Liver disease in adult transfusion-dependent beta-thalassaemic patients: investigating the role of iron overload and chronic HCV infection. PG - 420-7. Liver Int. 2013 Mar;33(3):420-7. doi: 10.1111/liv.12095.
4. Shokrgozar N., Amirian, N., Ranjbaran, R. et al. Evaluation of regulatory T cells frequency and FoxP3/GDF-15 gene expression in β-thalassemia major patients with and without alloantibody; correlation with serum ferritin and folate levels. Ann Hematol 99, 421–429 (2020).
5. Tereshchenko G.V., Krasilnikova M.V., Gvozdev A.A., Ibragimova D.I., Mitrofanova A.M., Lokhmatova M.E., Novichkova G.A. Assessment of the degree of iron accumulation in children with transfusion-dependent β-thalassemia // Hematology and transfusiology. - 2016. -T. 61. No. 1. -S. 42-48.
REFERENCES:
1. Ali & Shahramian, Iraj & Yaghoobi, Hajar & Naderi, Majid & Azizi, Hakim. (2017). The Role of Immune System in Thalassemia Major: A Narrative Review. Journal of Pediatrics Review. In Press. 10.5812/jpr.14508.
2. Azarkeivan A et. al, Correlation between Heart, Liver and Pancreas Hemosiderosis Measured by MRI T2* among Thalassemia Major Patients from Iran. Arch Iran Med. 2016 Feb;19(2):96-100. doi: 0161902/AIM.006.
3. Kountouras D, et al, Liver disease in adult transfusion-dependent beta-thalassaemic patients: investigating the role of iron overload and chronic HCV infection. PG - 420-7. Liver Int. 2013 Mar;33(3):420-7. doi: 10.1111/liv.12095.
4. Shokrgozar N., Amirian, N., Ranjbaran, R. et al. Evaluation of regulatory T cells frequency and FoxP3/GDF-15 gene expression in β-thalassemia major patients with and without alloantibody; correlation with serum ferritin and folate levels. Ann Hematol 99, 421–429 (2020).
5. Tereshchenko G.V., Krasilnikova M.V., Gvozdev A.A., Ibragimova D.I., Mitrofanova A.M., Lokhmatova M.E., Novichkova G.A. Assessment of the degree of iron accumulation in children with transfusion-dependent β-thalassemia // Hematology and transfusiology. - 2016. -T. 61. No. 1. -S. 42-48.
