The pathogenicity of von Willebrand factor in thrombotic thrombocytopenic purpura: reconsideration of treatment with cryopoor plasma.
The pathogenicity of von Willebrand factor in thrombotic thrombocytopenic purpura: reconsideration of treatment with cryopoor plasma.
复制标题
冯维勒布兰德因子在血栓性血小板减少性紫癜中的致病性：重新考虑低温血浆治疗。
DOI：
10.1111/j.1537-2995.2005.00674.x
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发表时间：
2006
期刊：
影响因子：
2.9
通讯作者：
Dwyre,DenisM
中科院分区：
文献类型：
--
作者：
Raife,ThomasJ;Friedman,KennethD;Dwyre,DenisM
在了解血栓性血小板减少性紫癜（TTP）的新发展提供了机会，以改善病人的护理。广泛认为TTP微血管血栓形成的历史模型涉及循环超大型血管性血友病因子（ULVWF）引起自发血小板（PLT）聚集。从这个致病模型来看，对用于治疗患者的新鲜冷冻血浆（FFP）中ULVWF的担忧导致了冷冻血浆（CPP）作为替代方案的广泛使用。然而，很少有证据表明循环ULVWF有助于TTP的微血管血栓形成。新的证据表明，TTP中PLT聚集体的形成可能在VWF从内皮细胞释放的过程中介导。此外，临床研究并未证明CPP在治疗TTP方面优于FFP。由于CPP可能降低了TTP治疗中重要因子的浓度，包括ADAMTS13金属蛋白酶，因此有必要重新评估CPP在TTP治疗中的应用。
New developments in the understanding of thrombotic thrombocytopenic purpura (TTP) provide opportunities for improved patient care. A widely held historical model of TTP microvascular thrombosis implicated circulating ultralarge von Willebrand factor (ULVWF) in causing spontaneous platelet (PLT) aggregation. From this pathogenic model, concerns about ULVWF in fresh‐frozen plasma (FFP) used to treat patients led to widespread use of cryopoor plasma (CPP) as an alternative. There is scant evidence, however, that circulating ULVWF contributes to microvascular thrombosis in TTP. New evidence suggests that the formation of PLT aggregates in TTP may be mediated by VWF in the process of being released from endothelium. Moreover, clinical studies do not demonstrate superior efficacy of CPP compared to FFP in the treatment of TTP. Because CPP may have reduced concentrations of factors important in the treatment of TTP, including ADAMTS13 metalloprotease, a reappraisal of the use of CPP in the treatment of TTP is warranted.