Thalassemia is a group of inherited blood disorders due to the reduction or absence of globin chain synthesis which can cause hemolytic anemia. β-thalassemia major is a severe type of thalassemia, in which patients require lifelong transfusions for survival. Extravascular hemolysis on the spleen results in splenomegaly, meanwhile, extramedullary hematopoiesis causing hypersplenism to develop in beta-thalassemia major patients. Hypersplenism, symptomatic splenomegaly, and increased annual need for blood transfusion in thalassemia patients are indicators of splenectomy. However, aside from the benefit of splenectomy which may reduce the need for transfusion in people with thalassemia with the goal of reducing iron overload, it may also be linked to several major negative effects that may outweigh any benefits, such as postoperative infections with high mortality rate and thrombotic events.
Thalassemia is a group of hereditary hemoglobin disorders characterized by insufficient production of at least one globin chain, resulting in unbalanced production of globin chains. Homozygous mutations in the -globin gene, resulting in the absence of the -chain, are the main cause of -thalassemia major. Because the -chain in -thalassemia major is not formed, there is an accumulation of free -chains in red blood cells, which can trigger apoptosis and hemolysis resulting in ineffective erythropoiesis. Management for -thalassemia major patients requires lifelong therapy with blood transfusions and medication. However, blood transfusions and routine administration of iron chelation drugs cannot cure -thalassemia major. Thalassemia can be cured through definitive therapies including bone marrow or stem cell transplantation and genetic therapy. Bone marrow transplantation is a treatment option for children and adolescents suffering from certain types of cancer and other blood disorders such as thalassemia.
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