Major surgery in haemophilic patients with inhibitors using recombinant factor VIIa.
Major surgery in haemophilic patients with inhibitors using recombinant factor VIIa.
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使用重组因子 VIIa 对血友病患者进行大手术。
DOI：
10.1159/000217252
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发表时间：
1996
期刊：
影响因子：
--
通讯作者：
M. Shuman
中科院分区：
文献类型：
--
作者：
J. Ingerslev;D. Freidman;D. Gastineau;G. Gilchrist;H. Johnsson;G. Lucas;J. Mcpherson;E. Preston;E. Scheibel;M. Shuman
在血友病患者中，抑制缺失的凝血因子功能的抗体的产生引起了几个微妙的问题。最重要的是，抗体会阻断特异性凝血因子的功能，并且抗体活性往往如此强烈，以至于有效的替代治疗被超越。因此，必须采取替代措施控制出血。在最常用的那些中，如因子IX浓缩物、活化的凝血酶原复合物浓缩物和猪源的因子VIII，新的重组活化因子VII分子已在临床上评价了几年，结果令人鼓舞。本论文的目的是介绍一系列患有血友病A或B的患者，这些患者的抑制剂使临床表现复杂化，并且需要手术治疗。作为这种基因工程因子VIIa生产商设计的同情使用计划的一部分，12名患者接受了挽救生命或必要的手术，其中重组因子VIIa产品用于促进13例外科手术中的止血。由于活化的因子VIIa的体内半衰期短，计划频繁给药，每2-3小时注射因子VIIa，持续2天，之后延长给药间隔。在1例病例中，未报告最终治疗结果的总体评价，但在所有其他12例病例中，最终结果被认为是极好（n = 11）或有效（n = 1）。在所有病例中，均不需要其他类型的凝血因子治疗方式。总之，重组凝血因子VIIa似乎是一个诱人的替代传统治疗血友病患者的抑制剂，其中手术是必要的。对于其他类型的止血剂，血友病抑制剂患者的手术研究很少，手术通常仅限于危及生命的情况。
In the haemophilic patient, development of antibodies that inhibit the function of the missing coagulation factor causes several delicate problems. Most importantly, antibodies will block the function of the specific coagulation factor, and often the antibody activity is so fierce that effective substitution therapy is outruled. In consequence, alternative measures must be adopted to control bleeding. Amongst those most commonly employed, like factor IX concentrates, activated prothrombin complex concentrates, and factor VIII of porcine origin, a new recombinant activated factor VII molecule has been evaluated clinically for some years with promising results. The aim of the present paper was to present a series of patients suffering from haemophilia A or B in whom inhibitors have complicated the clinical picture, and in whom a surgical procedure was indicated. As part of a compassionate use program devised by the producer of this genetically engineered factor VIIa, 12 patients underwent life-saving or essential surgery where the recombinant factor VIIa product was used to promote haemostasis in 13 surgical procedures. Due to a short in vivo half-life of activated factor VIIa, frequent administration was scheduled, injecting factor VIIa every 2-3 h for up to 2 days after which dosage intervals were prolonged. In one case, a global evaluation of the end treatment result was not reported, but in all of the other 12 cases the end result were considered excellent (n = 11) or efficient (n = 1). In none of the cases was other types of coagulation factor treatment modalities necessary. In conclusion, recombinant factor VIIa seems a tempting alternative to traditional treatment of the haemophilic patient with inhibitors, in whom surgery is called for. With other types of haemostatic agents, surgery in haemophilic inhibitor patients has only been studied rarely, and operations have generally been restricted to life-threatening situations.