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目的 动态测定血栓性血小板减少性紫癜 (TTP)患者发病过程中血管内皮细胞标志物———凝血酶调节蛋白(TM)、血管内皮生长因子(VEGF)的浓度、微血管病性溶血性贫血相关参数的变化,并分析其临床意义。方法 对 3例TTP患者进行了血管性血友病因子 (vWF)裂解蛋白酶活性分析,外周血涂片连续动态计数破碎红细胞百分率;用Western印迹分析结合灰度扫描测定血浆TM水平;血浆VEGF水平用ELISA法进行分析;对 1例存在视力损害的TTP患者行眼底血管荧光造影,以寻找微血管血栓形成的可能证据。结果 3例TTP患者均有vWF裂解不全,发病时外周血涂片破碎红细胞百分率分别为 1. 65%、2. 50%、3. 32%,均值为 2. 49%, 破碎红细胞百分率随病情好转而逐渐下降。TTP患者血浆TM和VEGF水平与正常人相比在发病时均明显升高,并与TTP病情严重程度存在相关性, 1例TTP患者眼底荧光造影存在眼底微小血管微血栓形成。结论 vWF裂解蛋白酶活性降低有助于TTP的肯定性诊断,血浆TM、VEGF水平以及外周血破碎红细胞百分率的动态测定有助于反映TTP患者病情严重程度及对治疗反应的评价。选择性眼底荧光造影对TTP微血管血栓形成的判定有一定的临床价值。
Objective To dynamically determine the levels of vascular endothelial cell markers, such as thrombin regulatory protein (TM), vascular endothelial growth factor (VEGF), and microangiopathy-related hemolytic anemia in patients with thrombotic thrombocytopenic purpura (TTP) Changes, and analyze its clinical significance. Methods Three cases of TTP patients were analyzed for vWF cleavage protease activity. Peripheral blood smears were continuously counted dynamically to break down the percentage of erythrocytes. Plasma levels of TM were measured by Western blot analysis combined with gray-scale scanning. ELISA analysis; one case of visual impairment TTP patients underwent fundus fluorescein angiography, in order to find possible evidence of microvascular thrombosis. Results 3 cases of TTP patients were vWF pyrolysis, the incidence of peripheral blood smear fragments were 1.65%, 2.50%, 3.32%, an average of 2.49%, the percentage of broken red blood cells improved with the condition And gradually decline. The levels of plasma TM and VEGF in patients with TTP were significantly higher at onset than those in normal subjects and correlated with the severity of TTP. One case of TTP had fundus microvascular thrombosis with fundus fluorescein angiography. Conclusions The reduction of vWF cleavage protease contributes to the positive diagnosis of TTP. The dynamic measurement of plasma TM and VEGF levels and the percentage of erythrocytes in peripheral blood can help to reflect the severity of TTP and the evaluation of response to treatment. Selective fundus fluorescein angiography on the determination of TTP microvascular thrombosis have some clinical value.