Plasma ADAMTS13, von Willebrand Factor (VWF) and VWF Propeptide Profiles in Patients with DIC and Related Diseases

Plasma ADAMTS13, von Willebrand Factor (VWF) and VWF Propeptide Profiles in Patients with DIC and Related Diseases
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DOI:
10.1016/j.thromres.2011.10.011
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发表时间:
2012-05-01
影响因子:
7.5
通讯作者:
Nobori, Tsutomu
Nobori, Tsutomu
中科院分区:
医学3区
文献类型:
--
作者:
Habe, Koji;Wada, Hideo;Nobori, Tsutomu

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ADAMTS13、内皮血管性血友病因子 (VWF) 和相关蛋白参与一些危及生命的全身性血栓性凝血病的发病机制。血栓性血小板减少性紫癜 (TTP) 中血浆 ADAMTS13 活性的变化是众所周知的,但也与脓毒症弥散性血管内凝血 (DIC) 相关。在此,我们研究了 69 例 DIC 患者、143 例非 DIC、21 例血栓性血小板减少性紫癜 (TTP) 和 23 例非典型溶血性尿毒症综合征 (aHUS) 患者的 ADAMTS13 活性、VWF 和 VWF 前肽 (VWFpp) 抗原,以诊断 DIC。 DIC 患者血浆 ADAMTS13 活性显着较低,血浆 VWF 和 VWFpp 水平这些患者的血浆 VWFpp 水平最高,但 DIC 患者与 aHUS 患者之间血浆 VWFpp 水平无显着差异。 DIC与非DIC患者血浆ADAMTS13活性、VWF和VWFpp抗原在感染性疾病和恶性疾病患者中存在显着差异,但VWFpp/VWF比值仅在感染性疾病患者中存在显着差异。作为预后指标,非幸存者的血浆 VWFpp 水平显着高于幸存者。那么,VWFpp/VWF比值和VWFpp/ADAMATS13比值将成为DIC的有效信息指标。这些结果表明ADAMTS13/VWF谱可能在DIC的发病机制中发挥重要作用,并且ADAMTS13和VWFpp是DIC诊断和预后的有用指标。 (c) 2011 Elsevier Ltd. 保留所有权利。
ADAMTS13, endothelial von Willebrand factor (VWF) and related proteins are involved in the pathogenesis of some life threatening systemic thrombotic coagulopathies. Changes of plasma ADAMTS13 activity in thrombotic thrombocytopenic purpura (TTP) is well known but is also involved in septic disseminated intravascular coagulation (DIC). Here we investigated the ADAMTS13 activity, VWF and VWF propeptide (VWFpp) antigens in 69 patients with DIC, 143 with non-DIC, 21 with thrombotic thrombocytopenic purpura (TTP) and 23 with atypical hemolytic uremic syndrome (aHUS) for diagnosis of DIC.The plasma ADAMTS13 activity was significantly low in patients with DIC, and the plasma levels of VWF and VWFpp antigens, were the highest in these patients, but there were no significant differences in the plasma VWFpp levels between the patients with DIC and those with aHUS. The difference in the plasma ADAMTS13 activity, the VWF and VWFpp antigens between DIC and non-DIC cases was significant in those with infectious and malignant diseases, but the difference in the VWFpp/VWF ratio were significant only in subjects with infectious diseases. As an indicator for prognosis, the plasma levels of VWFpp were significantly higher in non-survivors than in survivors. Then, VWFpp/VWF ratio and VWFpp/ADAMATS13 ratio will be potent informative indicators in DIC.These findings suggest that ADAMTS13/VWF profiles may have important roles in the pathogenesis of DIC, and that ADAMTS13 and VWFpp are useful indicators for the diagnosis and prognosis of DIC. (c) 2011 Elsevier Ltd. All rights reserved.