von Willebrand factor proteolysis is deficient in classic, but not in bone marrow transplantation-associated, thrombotic thrombocytopenic purpura.
von Willebrand factor proteolysis is deficient in classic, but not in bone marrow transplantation-associated, thrombotic thrombocytopenic purpura.
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冯维勒布兰德因子蛋白水解作用在经典的血栓性血小板减少性紫癜中存在缺陷，但在骨髓移植相关的血栓性血小板减少性紫癜中则没有。
DOI：
10.1182/blood.v93.11.3798
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发表时间：
1999
期刊：
影响因子：
20.3
通讯作者：
R. Fijnheer
中科院分区：
文献类型：
--
作者：
R. M. van der Plas;M. Schiphorst;E. Huizinga;R. Hené;L. Verdonck;J. Sixma;R. Fijnheer
骨髓移植后血栓性血小板减少性紫癜（TTP）的临床过程和治疗与经典TTP不同。经典TTP的特征是抑制特异性切割血管性血友病因子（vWF）的血浆蛋白酶，从而减少其多聚体大小。我们研究了这种蛋白酶是否也在BMT相关TTP中被抑制。来自经典或BMT相关TTP患者的血浆与重组vWF R834Q孵育，重组vWF R834Q是一种对蛋白酶敏感性增强的vWF突变体。在Western印迹上分析和定量vWF多聚体的蛋白水解。金属蛋白酶活性在经典TTP患者组中受到强烈抑制。然而，金属蛋白酶活性在BMT相关TTP患者组中是正常的。两组患者之间的活动差异非常显著（P = 0.0016）。结果表明，经典型TTP和BMT相关型TTP的病因确实不同，并为BMT相关型TTP中血浆置换缺乏成功提供了解释。
Thrombotic thrombocytopenic purpura (TTP) after bone marrow transplantation (BMT) differs from classic TTP in its clinical course and therapy. A characteristic of classic TTP is the inhibition of a plasma protease that specifically cleaves von Willebrand factor (vWF), thus reducing its multimeric size. We investigated whether this protease was also inhibited in BMT-associated TTP. Plasma from patients with classic or BMT-associated TTP was incubated with recombinant vWF R834Q, a vWF mutant with enhanced sensitivity to the protease. The proteolysis of vWF multimers was analyzed and quantified on Western blot. Metalloprotease activity was strongly inhibited in the classic TTP patient group. However, metalloprotease activity was normal in the BMT-associated TTP patient group. The difference in activity between the two patient groups was highly significant (P =.0016). The results indicate that the etiologies of classic and BMT-associated TTP are indeed different and provide an explanation for the lack of success of plasma exchange in BMT-associated TTP.
骨髓移植后溶血性尿毒症综合征。
影响因子：
4.8
作者：
Juckett,M;Perry,EH;Daniels,BS;Weisdorf,DJ
通讯作者：
Weisdorf,DJ