Kremer Hovinga J A, Zeerleder S, Kessler P, Romani de Wit T, van Mourik J A, Hack C E, ten Cate H, Reitsma P H, Wuillemin W A, Lämmle B
Department of Hematology and Central Hematology Laboratory, Inselspital, University of Bern, Bern, Switzerland.
J Thromb Haemost. 2007 Nov;5(11):2284-90. doi: 10.1111/j.1538-7836.2007.02743.x. Epub 2007 Aug 22.
Insufficient control of von Willebrand factor (VWF) multimer size as a result of severely deficient ADAMTS-13 activity results in thrombotic thrombocytopenic purpura associated with microvascluar thrombosis and platelet consumption, features not seldom seen in severe sepsis and septic shock.
ADAMTS-13 activity and VWF parameters of 40 patients with severe sepsis or septic shock were compared with those of 40 healthy controls of the same age and gender and correlated with clinical findings and sepsis outcome.
ADAMTS-13 activity was significantly lower in patients than in healthy controls [median 60% (range 27-160%) vs. 110% (range 63-200%); P < 0.001]. VWF parameters behaved reciprocally and both VWF ristocetin cofactor activity (RCo) and VWF antigen (VWF:Ag) were significantly (P < 0.001) higher in patients compared with controls. Neither ADAMTS-13 activity nor VWF parameters correlated with disease severity, organ dysfunction or outcome. However, a contribution of acute endothelial dysfunction to renal impairment in sepsis is suggested by the significantly higher VWF propeptide and soluble thrombomodulin levels in patients with increased creatinine values as well as by their strong positive correlations (creatinine and VWF propeptide r(s) = 0.484, P < 0.001; creatinine and soluble thrombomodulin r(s) = 0.596, P < 0.001).
VWF parameters are reciprocally correlated with ADAMTS-13 activity in severe sepsis and septic shock but have no prognostic value regarding outcome.
由于ADAMTS - 13活性严重缺乏导致血管性血友病因子（VWF）多聚体大小控制不足，会引发伴有微血管血栓形成和血小板消耗的血栓性血小板减少性紫癜，这些特征在严重脓毒症和脓毒性休克中并不少见。
将40例严重脓毒症或脓毒性休克患者的ADAMTS - 13活性和VWF参数与40例年龄和性别匹配的健康对照者进行比较，并与临床发现和脓毒症结局相关联。
患者的ADAMTS - 13活性显著低于健康对照者[中位数60%（范围27 - 160%）对110%（范围63 - 200%）；P < 0.001]。VWF参数呈现相反变化，与对照者相比，患者的VWF瑞斯托霉素辅因子活性（RCo）和VWF抗原（VWF:Ag）均显著更高（P < 0.001）。ADAMTS - 13活性和VWF参数均与疾病严重程度、器官功能障碍或结局无关。然而，肌酐值升高患者中VWF前体肽和可溶性血栓调节蛋白水平显著更高，以及它们之间的强正相关性[肌酐与VWF前体肽r(s) = 0.484，P < 0.001；肌酐与可溶性血栓调节蛋白r(s) = 0.596，P < 0.001]提示急性内皮功能障碍对脓毒症患者肾功能损害有影响。
在严重脓毒症和脓毒性休克中，VWF参数与ADAMTS - 13活性呈相反相关，但对结局无预后价值。
