Franchini Massimo, Zaffanello Marco, Lippi Giuseppe
Servizio di Immunoematologia e Medicina Trasfusionale, Dipartimento di Patologia e Medicina di Laboratorio, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy.
Blood Coagul Fibrinolysis. 2010 Oct;21(7):615-9. doi: 10.1097/MBC.0b013e32833c2bb5.
Owing to its ability to raise plasma levels of factor VIII and von Willebrand factor levels, the synthetic vasopressin analogue desmopressin has become the mainstay of treatment for type 1 von Willebrand disease and mild hemophilia A. A long clinical experience with this drug for prevention or treatment of bleedings in these patients has been accumulated over the past 30 years, supporting its hemostatic effectiveness and safety. In this paper, we summarize the current knowledge on the mechanisms of action as well as its biological effects in patients with mild hemophilia A. The results of the most important clinical trials in this setting are also reviewed.
由于合成血管加压素类似物去氨加压素能够提高血浆中凝血因子 VIII 水平和血管性血友病因子水平,它已成为 1 型血管性血友病和轻度甲型血友病治疗的主要药物。在过去 30 年里,积累了使用这种药物预防或治疗这些患者出血的长期临床经验,证明了其止血效果和安全性。在本文中,我们总结了目前关于去氨加压素在轻度甲型血友病患者中的作用机制及其生物学效应的知识。同时也回顾了该领域最重要的临床试验结果。