The value of anti-von Willebrand factor antibody in the mechanism of idiopathic thrombotic thrombocytopenic purpura
10.3760/cma.j.issn.1009-9158.2012.12.014
- VernacularTitle:抗血管性血友病因子抗体在特发性血栓性血小板减少性紫癜发病机制中的作用
- Author:
Jingyu ZHANG
;
Fang LIU
;
Zhenni MA
;
Ningzheng DONG
;
Jian SU
;
Yiming ZHAO
;
Fei SHEN
;
Anyou WANG
;
Changgeng RUAN
- Publication Type:Journal Article
- Keywords:
Purpura,thrombocytopenic,idiopathic;
von Willebrand factor;
Autoantibodies;
ADAM proteins
- From:
Chinese Journal of Laboratory Medicine
2012;(12):1118-1124
- CountryChina
- Language:Chinese
-
Abstract:
Objective This study is aimed at determining whether anti-von Willebrand factor (VWF) autoantibodies are present in the plasma of idiopathic thrombotic thrombocytopenic purpura (TTP) patients with normal ADAMTS13 activity and undetectable anti-ADAMTS13 antibodies,and at examining whether murine monoclonal antibodies (mAbs) against human VWF decrease the susceptibility of VWF to ADAMTS13 in vitro.Methods Anti-VWF autoantibodies and ultralarge VWF (UL-VWF) multimers were measured in plasma samples of 53 adult patients with idiopathic TTP by enzyme-linked immunosorbent assay and sodium dodecylsulphate-agarose gel electrophoresis,respectively.Moreover,the effects of eight murine mAbs to different human VWF domains on VWF cleavage by ADAMTS13 were evaluated under fluid shear stress and static/denaturing conditions,respectively.Results Anti-VWF antibodies and UL-VWF multimers were detected in two TTP patients with normal ADAMTS13 activity and undetectable anti-ADAMTS13antibodies.The SZ34,an anti-VWF mAb,inhibited VWF proteolysis mediated by ADAMTS13 under flow,but not static conditions.Conclusion Anti-VWF antibody may be one of the causes of idiopathic TTP with normal ADAMTS13 activity and undetectable anti-ADAMTS13 antibodies.
