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首页> 外文期刊>Blood: The Journal of the American Society of Hematology >At least Type VWD2B is a discrete variant of VWD, isn't it?
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At least Type VWD2B is a discrete variant of VWD, isn't it?

机译:至少VWD2B型是VWD的离散型,不是吗?

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Type 2B von Willebrand Disease (VWD2B) is generally characterized as including patients with bleeding symptoms whose laboratory studies demonstrate (1) reduced von Willebrand factor (VWF), (2) loss of high- molecular-weight VWF multimers, (3) thrombocytopenia, and (4) platelet aggregation of patient platelet-rich plasma to low concentrations of ristocetin (usually <= 0.6 mg/mL).~(1,2) The article by Federici et al is critical because it demonstrates the pheno-typic diversity of laboratory tests in patients diagnosed with VWD2B. While clinical laboratory studies using ristocetin have recognized laboratory to laboratory differences, this collaborative group includes laboratories of excellence that are recognized for their precision in VWD diagnosis
机译:2B型von Willebrand病(VWD2B)通常被表征为包括具有出血症状的患者,其实验室研究表明(1)von Willebrand因子(VWF)降低,(2)高分子量VWF多聚体丧失,(3)血小板减少, (4)患者富含血小板的血浆的血小板聚集至低浓度的瑞斯托霉素(通常<= 0.6 mg / mL)。〜(1,2)Federici等人的文章至关重要,因为它证明了血小板的表型多样性诊断为VWD2B的患者的实验室检查。尽管使用瑞斯托菌素的临床实验室研究已经认可了实验室与实验室之间的差异,但是这个协作小组包括了卓越的实验室,这些实验室因其VWD诊断的准确性而得到认可

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