DiMichele D M, Hathaway W E
Department of Pediatrics, University of Colorado School of Medicine, Denver.
Am J Hematol. 1990 Jan;33(1):39-45. doi: 10.1002/ajh.2830330108.
Twenty-one patients with prolonged bleeding times secondary to inherited disorders of platelet function and eight patients with prolonged bleeding times secondary to acquired platelet dysfunction were given 0.3 micrograms per kilogram of DDAVP, 1-deamino-8-D-arginine vasopressin, intravenously. Sixteen of twenty-two DDAVP trials in patients with inherited platelet dysfunction (73%) and seven of the nine DDAVP trials in patients with acquired platelet dysfunction (78%) resulted in normalization or shortening of the prolonged bleeding times by at least 4 min. The bleeding time response did not correlate with changes in the levels of von Willebrand factor (vWf) antigen or ristocetin cofactor activity, nor was it associated with changes in vWf multimeric analysis or in vitro platelet aggregations following the administration of DDAVP. Shortening of the bleeding time with DDAVP was seen in patients with a failure to release/storage pool type defect, thromboxane synthesis type defect, Bernard-Soulier syndrome, Glanzmann's thrombasthenia, the May-Hegglin anomaly, liver disease, nonuremic renal disease, myelofibrosis, and Tangier's disease.
21例因遗传性血小板功能障碍导致出血时间延长的患者和8例因获得性血小板功能障碍导致出血时间延长的患者,静脉注射每千克体重0.3微克的去氨加压素(1-去氨基-8-D-精氨酸血管加压素)。在遗传性血小板功能障碍患者的22次去氨加压素试验中,有16次(73%),在获得性血小板功能障碍患者的9次去氨加压素试验中,有7次(78%),导致延长的出血时间正常化或缩短至少4分钟。出血时间反应与血管性血友病因子(vWf)抗原水平或瑞斯托霉素辅因子活性的变化无关,也与去氨加压素给药后vWf多聚体分析或体外血小板聚集的变化无关。在存在释放/储存池型缺陷、血栓素合成型缺陷、伯-苏综合征、血小板无力症、梅-赫异常、肝病、非尿毒症性肾病、骨髓纤维化和丹吉尔病的患者中,观察到去氨加压素使出血时间缩短。