Gøtze Jens Peter, Hillarp Andreas, Lindblom Anders, Björk Peter, Strandberg Karin, Nielsen Lars Bo
Rigshospitalet, Klinisk Biokemisk Afdeling, Københavns Universitet, Biomedicinsk Institut, København Ø.
Ugeskr Laeger. 2008 Aug 11;170(33):2446-9.
Thrombotic microangiopathy can be caused by several conditions which are difficult to diagnose from the clinical presentation alone. Deficient enzyme activity of a newly-discovered enzyme, ADAMTS-13, can lead to thrombotic thrombocytopenic purpura (TTP). Lack of ADAMTS-13 activity causes increased concentrations of high molecular weight von Willebrand factor forms and increased platelet aggregation. Measurement of ADAMTS-13 activity is useful for the diagnosis of TTP and may also be relevant as a prognostic test for recurrent TTP.
血栓性微血管病可由多种病症引起，仅根据临床表现很难做出诊断。一种新发现的酶——金属蛋白酶去整合素样金属蛋白酶13（ADAMTS-13）的酶活性不足可导致血栓性血小板减少性紫癜（TTP）。ADAMTS-13活性缺乏会导致高分子量血管性血友病因子形式的浓度增加以及血小板聚集增加。检测ADAMTS-13活性有助于TTP的诊断，也可能作为复发性TTP的预后检测指标。
