蛋白C、蛋白S及抗凝血酶Ⅲ活性在β地中海贫血患者中检测的意义

Significance of detection of protein C,protein S and antithrombinⅢactivity in patients withβ-thalassemia

目的:探讨蛋白C(PC)、蛋白S(PS)、抗凝血酶Ш(AT-Ⅲ)在β地中海贫血(β地贫)中的意义。方法:选取2019年5-12月广西医科大学第一附属医院与广西医科大学第七附属医院收治的83例β地贫患者作为研究对象,另选择同期体检正常的48例正常人作为对照组,分别采用发色底物法和凝固法测定两组的PC、PS和AT-Ⅲ活性水平并进行比较,并分析PC、PS、AT-Ⅲ与血清铁蛋白、血小板(PLT)的相关性。结果:β地贫患者中PC、PS活性降低的比例分别是98.8%和86.7%。与对照组比较,中间型β地贫(β-TI)与重型β地贫(β-TM)的PC、PS活性水平均显著降低(P<0.001);β-TI患者的AT-Ⅲ活性低于对照组(P<0.05);脾切除组与非脾切除组比较,PC、PS、AT-Ⅲ活性比较,差异均无统计学意义(P>0.05);AT-Ⅲ与PC(r=0.298,P=0.006)、PS(r=0.412,P<0.001)均呈正相关关系。结论:β地中海贫血患者血浆中的PC、PS活性显著降低,为地贫患者存在慢性高凝状态提供证据,同时也为地中海贫血患者血栓形成提供了有利的监测指标。

Objective:To explore the significance of protein C(PC),protein S(PS)and antithrombinⅢ(AT-Ⅲ)inβ-thalassemia.Methods:Eighty-three patients withβ-thalassemia treated in The First Affiliated Hospital of Guangxi Medical University and Wuzhou Gongren Hospital in Guangxi from May 2019 to December 2019 were selected as the subjects,and 48 healthy persons with normal physical examination in the same period were selected as the control group.The activity levels of PC,PS and AT-Ⅲin the two groups were measured and compared by chromogenic substrate method and coagulation method,respectively.The correlations between PC,PS,AT-Ⅲand serum ferritin and platelet(PLT)were analyzed.Results:The decreased activities of PC and PS in patients withβ-thalassemia were 98.8%and 86.7%,respectively.Compared with the control group,the activity levels of PC and PS in patients with intermediateβ-thalassemia(β-TI)and severeβ-thalassemia(β-TM)were significantly decreased(P<0.001).The activity of AT-Ⅲin patients withβ-TI was lower than that in the control group(P<0.05).There was no significant difference in PC,PS and AT-Ⅲactivity between splenectomy group and non-splenectomy group(P>0.05).There were a positive correlation between AT-Ⅲand PC(r=0.298,P=0.006)or AT-Ⅲand PS(r=0.412,P<0.001).Conclusion:The activities of PC and PS in plasma of patients withβ-thalassemia are significantly decreased,which provides evidence for the existence of chronic hypercoagulable state in patients with thalassemia and also provides a favorable monitoring index for thrombosis in patients with thalassemia.