4.凝血与纤溶系统异常促血小板凝聚物质如血小板激活因子（PAF）、体内存在vWF-CP抗体使血管性血友病因子（vWF）多聚体异常增多；血小板释放产物如β-血栓球蛋白（β-TG）等增加；内皮细胞释放组织因子，激活凝血系统，微血栓广泛形成；纤溶破坏，D-二聚体和纤溶酶原激活物抑制因子（plasminogenactivatorinhibitor，PAI）降低。
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