Molvarec Attila, Rigó János, Bõze Tamás, Derzsy Zoltán, Cervenak László, Makó Veronika, Gombos Tímea, Udvardy Miklós László, Hársfalvi Jolán, Prohászka Zoltán
Hangya lépcsõ 9, Budapest, H-1121, Hungary.
Thromb Haemost. 2009 Feb;101(2):305-11.
The activity of ADAMTS13, the von Willebrand factor (VWF) cleaving protease is low in several conditions, including HELLP (haemolysis, elevated liver enzymes, and low platelet count) syndrome. As HELLP syndrome develops in most cases on the basis of preeclampsia, our aim was to determine whether plasma ADAMTS13 activity is decreased in preeclampsia. Sixty-seven preeclamptic patients, 70 healthy pregnant women and 59 healthy non-pregnant women were involved in this case-control study. Plasma ADAMTS13 activity was determined with the FRETS-VWF73 assay, while VWF antigen (VWF:Ag) levels with an enzyme-linked immunosorbent assay. The multimeric pattern of VWF was analyzed by SDS-agarose gel electrophoresis. There was no significant difference in plasma ADAMTS13 activity between the preeclamptic and the healthy pregnant and non-pregnant groups (median [25-75 percentile]: 98.8 [76.5-112.8] %, 96.3 [85.6-116.2] % and 91.6 [78.5-104.4] %, respectively; p > 0.05). However, plasma VWF:Ag levels were significantly higher in preeclamptic patients than in healthy pregnant and non-pregnant women (187.1 [145.6-243.1] % versus 129.3 [105.1-182.8] % and 70.0 [60.2-87.3] %, respectively; p < 0.001). The multimeric pattern of VWF was normal in each group. Primiparas had lower plasma ADAMTS13 activity than multi-paras (92.6 [75.8-110.6] % versus 104.2 [92.1-120.8] %; p = 0.011). No other relationship was found between clinical characteristics, laboratory parameters and plasma ADAMTS13 activity in either study group. In conclusion, plasma ADAMTS13 activity is normal in preeclampsia despite the increased VWF:Ag levels. However, further studies are needed to determine whether a decrease in plasma ADAMTS13 activity could predispose preeclamptic patients to develop HELLP syndrome.
ADAMTS13(血管性血友病因子裂解蛋白酶)的活性在包括HELLP(溶血、肝酶升高和血小板减少)综合征在内的多种情况下较低。由于大多数HELLP综合征是在子痫前期的基础上发展而来,我们的目的是确定子痫前期患者血浆中ADAMTS13活性是否降低。本病例对照研究纳入了67例子痫前期患者、70例健康孕妇和59例健康非孕妇。采用FRETS-VWF73检测法测定血浆ADAMTS13活性,采用酶联免疫吸附测定法测定血管性血友病因子抗原(VWF:Ag)水平。通过SDS-琼脂糖凝胶电泳分析VWF的多聚体模式。子痫前期组与健康孕妇组和健康非孕妇组的血浆ADAMTS13活性无显著差异(中位数[25-75百分位数]:分别为98.8[76.5-112.8]%、96.3[85.6-116.2]%和91.6[78.5-104.4]%;p>0.05)。然而,子痫前期患者的血浆VWF:Ag水平显著高于健康孕妇和健康非孕妇(分别为187.1[145.6-243.1]%、129.3[105.1-182.8]%和70.0[60.2-87.3]%;p<0.001)。每组VWF的多聚体模式均正常。初产妇的血浆ADAMTS13活性低于经产妇(92.6[75.8-110.6]%对104.2[92.1-120.8]%;p=0.011)。在任何一个研究组中,均未发现临床特征、实验室参数与血浆ADAMTS13活性之间存在其他关联。总之,尽管VWF:Ag水平升高,但子痫前期患者的血浆ADAMTS13活性正常。然而,需要进一步研究以确定血浆ADAMTS13活性降低是否会使子痫前期患者易患HELLP综合征。