Galbusera M, Ruggenenti P, Noris M, Burnouf-Radosevich M, Benigni A, Mannucci P M, Remuzzi G
Mario Negri Institute for Pharmacological Research, Bergamo, Italy.
Lancet. 1995 Jan 28;345(8944):224-5. doi: 10.1016/s0140-6736(95)90224-4.
In thrombotic thrombocytopenic purpura (TTP), activated leucocytes release elastase which mobilises unusually large von Willebrand factor (vWF) multimers from the endothelium. We investigated the effect of an inhibitor of leucocyte elastase (alpha 1-antitrypsin) on circulating vWF and platelet count in a patient with chronic relapsing TTP. alpha 1-antitrypsin consistently normalised vWF multimeric composition but failed to increase platelet count and induce remission. Plasma cryosupernatant, which never normalised vWF, always induced laboratory and clinical remission within 96 hours. In chronic relapsing TTP, unusually large vWF multimers are not the main cause of intravascular platelet aggregation.
在血栓性血小板减少性紫癜（TTP）中，活化的白细胞释放弹性蛋白酶，该酶可从内皮细胞中动员出异常大的血管性血友病因子（vWF）多聚体。我们研究了白细胞弹性蛋白酶抑制剂（α1-抗胰蛋白酶）对一名慢性复发性TTP患者循环vWF和血小板计数的影响。α1-抗胰蛋白酶可使vWF多聚体组成持续恢复正常，但未能增加血小板计数并诱导缓解。血浆冷上清液虽从未使vWF恢复正常，但总能在96小时内诱导实验室指标和临床缓解。在慢性复发性TTP中，异常大的vWF多聚体并非血管内血小板聚集的主要原因。
