论文部分内容阅读
血管性假血友病(VW病)系常染色体遗传性凝血因子Ⅷ分子异常症。根据遗传形式,因子Ⅷ凝血活性(ⅧC),因子Ⅷ相关抗原(ⅧR∶Ag),因子Ⅷ瑞斯托霉素辅因子(ⅧR∶RCo)及瑞斯托霉素聚集等检查而分成多种亚型。近年用十二烷基磺酸钠(SDS)琼脂糖凝胶电泳发现血浆中ⅧR∶Ag分子量90万-2000万存在多种多聚体,供作VW病亚型的分型。纯合子和双重杂合子VW病,重症病例基本上不存在ⅧR∶Ag,Ⅰ型VW病ⅧRAg的多聚体总量减少。Ⅱ型VW病缺乏大的多聚体。据ⅢB VW病亚型在瑞斯托霉素作用下ⅧR∶Ag同血小板的反应亢进可资
Vasculogenic haemophilia (VW disease) is an autosomal genetic factor â ... § molecular abnormalities. According to the genetic form, factor Ⅷ coagulation activity (Ⅷ C), factor Ⅷ related antigen (Ⅷ R: Ag), factor Ⅷ ristocetin cofactor (Ⅷ R: RCo) and ristocetin aggregation and other tests are divided into a variety of sub type. In recent years, sodium dodecyl sulfate (SDS) agarose gel electrophoresis found in plasma Ⅷ R: Ag molecular weight 900000 -2000 million there are a variety of polymers for VW disease subtype. Homozygous and double heterozygous VW disease, severe cases, there is basically no Ⅷ R: Ag, Ⅰ type VW disease Ⅷ RAg total polymer decreased. Type II VW lacks large multimers. According to Ⅲ B VW disease subtype in the impact of ristocetin Ⅷ R: Ag with platelet hyperactivity can be funded