血管性血友病因子裂解酶(vWF-cp)可将大分子量的血管性血友病因子(vWF)多聚体水解成为生理状态下的小分子肽段。vWF-cp活性的异常或vWF对该酶敏感性的增加均影响vWF多聚体的降解,改变vWF与血小板和内皮下胶原的结合能力,易于导致血栓性血小板减少性紫癜、肿瘤的浸润与转移、血栓形成以及出血等多种病理状态的发生。本文就血浆vWF-cp的活性水平与上述病理状态的关系作一综述。 VWF lytic enzyme (vWF-cp) hydrolyzes large molecular weight vWF multimers to small molecule peptides in physiological state. Abnormality of vWF-cp activity or increased sensitivity of vWF to this enzyme affects the degradation of vWF multimers, alters the binding capacity of vWF to platelets and sub-collagen, and tends to cause thrombotic thrombocytopenic purpura, invasion and metastasis of tumors , Thrombosis and bleeding and other pathological conditions. In this paper, the relationship between plasma vWF-cp activity and the above pathological status is reviewed.