Su Xie, Liu Hui, Sun Na, Cheng Peng, Deng Dong-Hong, Long Yuan
Department of Hematology, The First Affiliated Hospital of Guangxi Medical University, Nanning 530021, Guangxi Zhuang Autonomous Region, China.
Department of Hematology, The First Affiliated Hospital of Guangxi Medical University, Nanning 530021, Guangxi Zhuang Autonomous Region, China,E-mail:
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2019 Apr;27(2):539-544. doi: 10.19746/j.cnki.issn.1009-2137.2019.02.037.
To explore whether the high risk factors possibly leading to hypercoagulative status and thrombosis exist in Thalassemia patients of Guangxi region through detecting plasma tissne factor-bearing microparticles (TFMP), procoagulatima activity, coagulation and anticoagulation function, fibrinolytic function, endothelial function and platelet count.
The TFMP procoagulation activity was detected by chromogenic saubstract method, the levels of tissue factors (TF), tissue factor pathway inhibitor(TFPI), protein C (PC), protein S (PS), antithrombin Ⅲ(AT-Ⅲ), tissue plasminogen activator (tPA), thrombin-activated fibrinolysis inhibitor (TAFI), soluble E-selectin (sE-sel), intercellular adhesion molecule-1 (ICAM-1) and thrombomodulin (TM) were detected by ELISA in thalassemia group (n=71) and control group (n=20 heathy persons).
Compared with control group, the AT-Ⅲ level decreased in β-thalastemia major group (TM) (P<0.05), the AT-Ⅲ level in TM group independeutly posstiody correlated with plt count (r=0.37, P<0.05); the levels of TF and sICAM in α-thalassenia intermediate group (TA) significantly decteased (P<0.05), the procoagulatim activity of TFMP in β-thalassemia intermediate group (TI) increased sngnificantly (P<0.05)， moreover positively corretated with AT-Ⅲ level (r=0.77, P<0.05). The TF and sICAM-1 levels in normal liver functim group of Thalassemia patients were lower tham those in control group (P<0.01 and P<0.05, respectively), the TFMP activity between normal and abnormal liver function was significantly different (P<0.05), while there were no significant difference in other correspoding indexes beween thalassemia group and control group as well as between each thalassemia groups.
The damage of liver function and reduction of anticoagylation substances exist in patients with β-thalassenia major in Guangxi region, the procoagulation activity of plasma TFMP in patients with β-thalassemia intermedia abnormally increases. All the above-mentioned factors may increase the risk of high coagulation status or thrombosis is thalassemia patients, the decrease of TF and SICAM-1 levels in patients with α-thalassemia intermedia may be factor against thrombosis.
通过检测广西地区地中海贫血患者血浆组织因子微粒（TFMP）、促凝活性、凝血与抗凝功能、纤溶功能、内皮功能及血小板计数，探讨是否存在可能导致高凝状态及血栓形成的高危因素。
采用发色底物法检测TFMP促凝活性，采用ELISA法检测地中海贫血组（n = 71）和对照组（20名健康人）的组织因子（TF）、组织因子途径抑制物（TFPI）、蛋白C（PC）、蛋白S（PS）、抗凝血酶Ⅲ（AT - Ⅲ）、组织型纤溶酶原激活剂（tPA）、凝血酶激活的纤溶抑制物（TAFI）、可溶性E选择素（sE - sel）、细胞间黏附分子 - 1（ICAM - 1）及血栓调节蛋白（TM）水平。
与对照组相比，重型β地中海贫血组（TM）的AT - Ⅲ水平降低（P < 0.05），TM组的AT - Ⅲ水平与血小板计数呈独立正相关（r = 0.37，P < 0.05）；中间型α地中海贫血组（TA）的TF及sICAM水平显著降低（P < 0.05），中间型β地中海贫血组（TI）的TFMP促凝活性显著升高（P < 0.05），且与AT - Ⅲ水平呈正相关（r = 0.77，P < 0.05）。地中海贫血患者肝功能正常组的TF及sICAM - 1水平低于对照组（分别为P < 0.01和P < 0.05），肝功能正常与异常组的TFMP活性差异有统计学意义（P < 0.05），而地中海贫血组与对照组以及各地中海贫血组之间的其他相应指标差异无统计学意义。
广西地区重型β地中海贫血患者存在肝功能损害及抗凝物质减少，中间型β地中海贫血患者血浆TFMP促凝活性异常升高。上述因素可能增加地中海贫血患者高凝状态或血栓形成的风险，中间型α地中海贫血患者TF及SICAM - 1水平降低可能是抗血栓形成的因素。
