CAJ | 학술논문

目的：探讨抗血管性血友病因子（vWF）抗体在特发性血栓性血小板减少性紫癜（TTP）发病中的意义。方法选择广西壮族自治区人民医院2010年1月至2013年6月间收治的特发性T T P患者28例，同时选取同期门诊体检健康者30例作为健康对照组，检测两组对象血浆抗血管性血友病因子裂解酶（ADAMTS13）和vWF相关指标。结果（1）28例特发性 TTP患者中有3例患者vWF抗体阳性（vWF抗体阳性组），其余患者 vWF抗体阴性（vWF抗体阴性组）。vWF抗体阳性组血浆 AD‐AM TS13抗体均为阴性。（2） vWF抗体阳性组血浆 ADAM TS13含量明显低于vWF抗体阴性组和健康对照组，3组血浆AD‐AM TS13水平比较差异有统计学意义（P＜0．01）。vWF抗体阳性组血浆vWF抗体 A值明显高于vWF抗体阴性组和健康对照组比较，差异有统计学意义（P＜0．01）。（3）28例特发性TTP经过血浆置换（PE）后ADAMTS13抗原含量明显升高，vWF抗体A值明显降低（P＜0．05）。结论 vWF抗体在特发性TTP发病中起到重要的作用，vWF抗体可能通过影响患者血浆ADAMTS13，促进vWF复合物形成，影响疾病发生。
목적：탐토항혈관성혈우병인자（vWF）항체재특발성혈전성혈소판감소성자전（TTP）발병중적의의。방법선택엄서장족자치구인민의원2010년1월지2013년6월간수치적특발성T T P환자28례，동시선취동기문진체검건강자30례작위건강대조조，검측량조대상혈장항혈관성혈우병인자렬해매（ADAMTS13）화vWF상관지표。결과（1）28례특발성 TTP환자중유3례환자vWF항체양성（vWF항체양성조），기여환자 vWF항체음성（vWF항체음성조）。vWF항체양성조혈장 AD‐AM TS13항체균위음성。（2） vWF항체양성조혈장 ADAM TS13함량명현저우vWF항체음성조화건강대조조，3조혈장AD‐AM TS13수평비교차이유통계학의의（P＜0．01）。vWF항체양성조혈장vWF항체 A치명현고우vWF항체음성조화건강대조조비교，차이유통계학의의（P＜0．01）。（3）28례특발성TTP경과혈장치환（PE）후ADAMTS13항원함량명현승고，vWF항체A치명현강저（P＜0．05）。결론 vWF항체재특발성TTP발병중기도중요적작용，vWF항체가능통과영향환자혈장ADAMTS13，촉진vWF복합물형성，영향질병발생。
Objective To discuss the effect of von willebrand factor (vWF) antibody on idiopathic thrombotic thrombocytopenic purpura(TTP) .Methods 28 cases of idiopathic thrombotic thrombocytopenic purpura were selected in our hospital from January 2011 to June 2013 ,and 30 healthy persons as control group ,ADAMTS13 and vWF of two groups were detected .Results 3 cases of patients were detected with positive vWF antibody ,vWF antibody negative in the remaining patients .3 patients with positive vWF antibody ADAMTS13 antibodies were negative ,ADAMTS13 levels were lower than the normal value .The levels of ADAMTS13 in vWF antibody positive patients was significantly lower than that of vWF antibody negative patients and the control group ,(P<0 .01) .vWF antibody positive patients plasma vWF antibody A was higher than vWF antibody negative and the control group (P<0 .01) .In idiopathic TTP after PE ADAMTS13 antigen increased significantly ,vWF antibody and A levels decreased significantly (P<0 .05) .Conclusion vWF antibodies in idiopathic thrombotic thrombocytopenic plays an important role in the pathogenesis of purpura ,vWF antibody may affect patients ADAMTS13 ,promote vWF complex formation ,effect of disease .