目前认为其为常染色体隐性遗传，系血管性血友病因子裂解蛋白酶（vonWillebrandfactorcleavingprotease，vWF-CP又称ADAMTS-13，adisintegrinmetalloproteinasewiththrombospondintypeImotif，member13）重度缺乏，导致vWF多聚体增多，损伤内皮细胞。
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