title: Normal hemostatic parameters in children and young adults with α-thalassemia diseases.
abstract: Thalassemia intermedia (TI), a non-transfusion dependent thalassemia, is divided into α-thalassemia, such as HbH disease, and β-thalassemia diseases, such as HbE/β+thromboembolism (TE) in TI has been mostly reported in β-thalassemia diseases with incidence rates of 3.9-29%. The present study enrolled 60 patients with α-thalassemia intermedia. The control groups were thalassemia major (TM) consisting of 17 patients diagnosed with β-thalassemia diseases, 24 patients diagnosed with splenectomized β-thalassemia diseases and 25 normal subjects. The mean±SD ages were 12.9±5.3, 15.0±3.8, 15.7±4.1 and 12.3±2.5years respectively. The coagulation markers in α-thalassemia patients, including D-dimer, thrombin-antithrombin complex (TAT) and prothrombin fragment (F1.2), were not significantly different compared to the levels in normal subjects. Similar results were found for the thromboelastometry, which is a method to assess global hemostasis involving the functions of coagulation and anticoagulation proteins, fibrinolysis and platelets. The hypercoagulability could be demonstrated in TM by high TAT in severe β-thalassemia patients and high TAT and D-dimer, shortened CT and CFT, high alpha angle, A20 and MCF only in the splenectomized β-thalassemia patients.
authors: Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. Electronic address: nongnuch.sir@mahidol.ac.th.
authors: Nongnuch
authors: N
authors: Sirachainan
authors: Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors: Ampaiwan
authors: A
authors: Chuansumrit
authors: Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors: Praguywan
authors: P
authors: Kadegasem
authors: Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors: Werasak
authors: W
authors: Sasanakul
authors: Division of Pediatric Nursing, Nursing Department, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors: Pakawan
authors: P
authors: Wongwerawattanakoon
authors: Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors: Lalita
authors: L
authors: Mahaklan
mesh_terms: D000293:Adolescent; D002648:Child; D005260:Female; D006487:Hemostasis / Q000502:physiology*; D006801:Humans; D008297:Male; D013916:Thrombelastography / Q000379:methods*; D017085:alpha-Thalassemia / Q000097:blood*
keywords: Hemostasis; Thromboelastometry; α-Thalassemia disease