血管性血友病是临床上常见的一种遗传性出血性疾病,其发病机制是患者的血管性血友病因子(vonWillebrandfactor,vWF)基因突变,导致血浆vWF数量减少或质量异常。国外报道vWD发病率约为千分之一。我国尚无统计学资料。由于vWD的类型不同,临床出血表现相差很大,实验室检查比血友病复杂,轻症患者的变化不典型,常需结合病史与临床综合判断。...
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