title: Hemostatic and thrombotic markers in patients with hemoglobin E/beta-thalassemia disease.
abstract: Increased frequency of thrombosis has been observed in patients with hemoglobin E/beta-thalassemia (Hb E/beta-thal) disease, particularly those who have previously been splenectomized. We compared various hemostatic and thrombotic markers in blood from 15 Hb E/beta-thal patients who were not splenectomized (NS), 15 who had been splenectomized (S), and 15 normal controls (NC). Levels of plasma thrombin-antithrombin, beta2 thromboglobulin, C-reactive protein, tissue plasminogen activator antigen were significantly higher in the S group than in either the NS or the NC groups, and levels of prothrombin fragment 1.2 were significantly higher in the S than in the NC group. Levels of plasminogen activator inhibitor-1 antigen were significantly higher in the S than in the NS group. Levels of protein C, protein S, antithrombin, and fibrinogen were significantly lower in the S and NS groups than in the NC group. Plasma lipoprotein(a) levels in the S and NS groups were not statistically different from NC. Our findings indicated that there is evidence of chronic low-grade coagulation and platelet activation, chronic low-grade inflammation, endothelial cell injury, impaired fibrinolysis, and decreased naturally occurring anticoagulants in splenectomized Hb E/beta-thal patients. These changes may account for the increased risk of thrombosis in these patients.
authors.affiliation: Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. rapac@mahidol.ac.th
authors.forename: Pantep
authors.initials: P
authors.lastname: Angchaisuksiri
authors.forename: Vichai
authors.initials: V
authors.lastname: Atichartakarn
authors.forename: Katcharin
authors.initials: K
authors.lastname: Aryurachai
authors.forename: Napaporn
authors.initials: N
authors.lastname: Archararit
authors.forename: Suporn
authors.initials: S
authors.lastname: Chuncharunee
authors.forename: Arjit
authors.initials: A
authors.lastname: Tiraganjana
authors.forename: Sasivimol
authors.initials: S
authors.lastname: Rattanasiri
mesh_terms: D000293:Adolescent; D000328:Adult; D019774:Blood Coagulation Factor Inhibitors / Q000032:analysis*; D001779:Blood Coagulation Factors / Q000032:analysis*; D016022:Case-Control Studies; D005260:Female; D006446:Hemoglobin E; D006487:Hemostasis / Q000502:physiology*; D006801:Humans; D008297:Male; D013156:Splenectomy; D017086:beta-Thalassemia / Q000097:blood* / Q000601:surgery
chemical_list: D019774:Blood Coagulation Factor Inhibitors; D001779:Blood Coagulation Factors; D006446:Hemoglobin E