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用于治疗血栓性血小板减少性紫癜的血液成分中血管性血友病因子抗原、血管性血友病因子裂解蛋白酶和蛋白S的比较。

Comparison of von Willebrand factor antigen, von Willebrand factor-cleaving protease and protein S in blood components used for treatment of thrombotic thrombocytopenic purpura.

作者信息

Yarranton H, Lawrie A S, Purdy G, Mackie I J, Machin S J

机构信息

Haemostasis Research Unit, Department of Haematology, University College London, London, UK.

出版信息

Transfus Med. 2004 Feb;14(1):39-44. doi: 10.1111/j.0958-7578.2004.00478.x.

DOI:10.1111/j.0958-7578.2004.00478.x
PMID:15043592
Abstract

Replacement of normal levels of von Willebrand factor-cleaving protease (VWF:CP, ADAMTS13) activity from infused plasma is important in plasma exchange (PEX) for the treatment of thrombotic thrombocytopenic purpura (TTP) patients. We have studied the VWF:CP activity, VWF multimer distribution, VWF:Ag, protein S (PS) activity and free PS antigen levels in fresh frozen plasma (FFP), cryosupernatant (CSP) and virally inactivated components treated with methylene blue/light (MB) or solvent detergent (SD) processes. VWF:CP activity was normal in all components tested and was retained following overnight storage at room temperature. CSP and SD plasma contained reduced levels of the highest molecular weight VWF multimers. Protein S activity was reduced below the normal range in SD plasma, but within the normal range for the other components tested. Virally inactivated SD- and MB-treated plasma may be an effective alternative to FFP and CSP in PEX for TTP. Reduced PS activity in SD plasma may predispose to venous thromboembolism, especially if infused in large volumes.

摘要

在血浆置换(PEX)治疗血栓性血小板减少性紫癜(TTP)患者时,从输注血浆中补充正常水平的血管性血友病因子裂解蛋白酶(VWF:CP,ADAMTS13)活性很重要。我们研究了新鲜冰冻血浆(FFP)、冷上清液(CSP)以及经亚甲蓝/光照(MB)或溶剂去污剂(SD)处理的病毒灭活成分中的VWF:CP活性、VWF多聚体分布、VWF:Ag、蛋白S(PS)活性和游离PS抗原水平。在所有测试成分中,VWF:CP活性均正常,且在室温下过夜储存后仍保持。CSP和SD血浆中最高分子量的VWF多聚体水平降低。SD血浆中的蛋白S活性降至正常范围以下,但其他测试成分中的蛋白S活性在正常范围内。在TTP的PEX中,经病毒灭活的SD和MB处理血浆可能是FFP和CSP的有效替代品。SD血浆中PS活性降低可能易引发静脉血栓栓塞,尤其是大量输注时。

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