Thromboembolic Complications in Splenectomized Patients with Dominantly Inherited beta-Thalassemia

摘要

The beta-thalassemias are a heterogenous group of inherited hemoglobin (Hb) disorders characterized by a reduced synthesis of beta-globin chains due to the mutated gene, and are frequently found in Mediterranean, African or Southeast Asian ancestry. beta-Thalassemia major (beta-TM) or intermedia (beta-TI) patients show severe to moderate anemia with ineffective erythropoiesis and he-molysis, and carry homozygous or compound heterozygous mutations. The heterozygote shows minor/trait phenotype with microcytosis, and absent or negligible hemolysis. Meanwhile, a fraction of heterozygotes with enhanced severity are classified as dominantly inherited beta-thalassemia, mostly arising from the mutation of exon 3 of the beta-globin gene. It is called Heinz body thalas-semia with a disease continuity of unstable Hb disease. Splenectomy is not usually recommended for beta-TM/TI patients because of the limited improvement of anemia and jaundice, along with the increased risk of thromboembolic events. Dominant beta-thalassemia patients occasionally require splenectomy for hypersple-nism; however, there is little information on the risk of splenectomy