Normal hemostatic parameters in children and young adults with alpha-thalassemia diseases

摘要

Thalassemia intermedia (TI), a non-transfusion dependent thalassemia, is divided into alpha-thalassemia, such as HbH disease, and beta-thalassemia diseases, such as HbE/beta(+) thromboembolism (TE) in TI has been mostly reported in rr-thalassemia diseases with incidence rates of 3.9-29%. The present study enrolled 60 patients with alpha-thalassemia intermedia. The control groups were thalassemia major (TM) consisting of 17 patients diagnosed with beta-thalassemia diseases, 24 patients diagnosed with splenectomized beta-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 +/- 2.5 years respectively. The coagulation markers in alpha-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 beta-thalassemia patients and high TAT and D-dimer, shortened CT and CFT, high alpha angle, A20 and MCF only in the splenectomized beta-thalassemia patients. (C) 2016 Elsevier Ltd. All rights reserved.