Schwartz R S, Bauer K A, Rosenberg R D, Kavanaugh E J, Davies D C, Bogdanoff D A
Department of Clinical Research, Cutter Biological, Miles Inc., Berkeley, California 94710.
Am J Med. 1989 Sep 11;87(3B):53S-60S. doi: 10.1016/0002-9343(89)80533-9.
Phase I clinical studies of antithrombin III (ATIII) concentrate demonstrated a mean in vivo incremental recovery of functional activity of 1.4 percent per unit/kg administered, an initial 50 percent disappearance time of 22 hours, and a biologic half-life of 3.8 days. Based on these observations, a treatment regimen designed to maintain plasma ATIII levels between 75 and 120 percent of normal has been developed. None of 10 subjects with congenital ATIII deficiency treated prophylactically had evidence of thromboembolism, including four pregnant women at the time of delivery. Five subjects treated for acute thrombosis and/or thromboembolism, four of whom were pregnant, recovered without further thrombotic extension or recurrence. Heparin resistance was reversed in two subjects, both pregnant. Nine subjects with acquired ATIII deficiency also received ATIII treatment for venous or arterial thrombosis or disseminated intravascular coagulation, all with low plasma ATIII levels. Two subjects with disseminated intravascular coagulation demonstrated improvement, one clinically, the other biochemically. All patients with congenital ATIII deficiency survived, but only five of nine with acquired deficiency survived, highlighting the importance in acquired ATIII deficiency of the underlying disease in prognosis. Survival rate was especially poor in subjects with arterial thrombosis in the setting of low plasma ATIII. Administration of ATIII concentrate was well tolerated. None of the subjects who received ATIII concentrate demonstrated evidence of an infectious transmissible agent. These studies demonstrate that it is now feasible to safely replace the deficient protein in congenital ATIII deficiency, either prophylactically or therapeutically.
抗凝血酶III(ATIII)浓缩剂的I期临床研究表明,每单位/千克给药后,体内功能活性的平均增量恢复为1.4%,初始50%消失时间为22小时,生物半衰期为3.8天。基于这些观察结果,已制定了一种治疗方案,旨在将血浆ATIII水平维持在正常水平的75%至120%之间。10名接受预防性治疗的先天性ATIII缺乏症患者中,无一例有血栓栓塞的证据,包括4名分娩时的孕妇。5名接受急性血栓形成和/或血栓栓塞治疗的患者,其中4名是孕妇,均康复且无进一步的血栓扩展或复发。两名孕妇患者的肝素抵抗得到了逆转。9名获得性ATIII缺乏症患者也因静脉或动脉血栓形成或弥散性血管内凝血接受了ATIII治疗,所有患者血浆ATIII水平均较低。两名弥散性血管内凝血患者有改善,一名在临床方面,另一名在生化方面。所有先天性ATIII缺乏症患者均存活,但9名获得性缺乏症患者中只有5名存活,这突出了在获得性ATIII缺乏症中基础疾病对预后的重要性。血浆ATIII水平低的动脉血栓形成患者的存活率尤其低。ATIII浓缩剂的给药耐受性良好。接受ATIII浓缩剂的患者均未显示有传染性传播因子的证据。这些研究表明,现在无论是预防性还是治疗性地安全替代先天性ATIII缺乏症中的缺陷蛋白都是可行的。