Factor VIIa in the treatment of haemophilia.
Factor VIIa in the treatment of haemophilia.
复制标题
治疗血友病的因子 VIIa。
DOI：
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发表时间：
1990
影响因子：
1.1
通讯作者：
U. Hedner
中科院分区：
文献类型：
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作者：
U. Hedner
重组FVIIa正在开发中，用于治疗FVIII/FIX抗体阳性的血友病患者。RFVIIa在血友病A和B犬以及20名血友病患者(1名血友病B和19名血友病A患者)中显示出止血活性。13例患者接受了危及生命的出血治疗，9例患者在手术中接受治疗(剂量：60-90微克/公斤，q3-4h)。1例患者在rFVIIa作为唯一凝血因子的覆盖下进行了膝关节滑膜切除术，没有任何问题。1例FXI缺乏症患者在切除术中获得成功治疗。血友病B患者接受完全止血的间隔室综合征(外科筋膜切开术)治疗。后来，他平安无事地接受了皮肤移植。治疗了两例中枢神经系统出血，一例严重的口腔出血，以及一例使用获得性FVIII抑制剂的患者的广泛鼻咽出血。凝血酶原时间缩短，APTT缩短。未观察到任何副作用。推测FVIIa不仅与组织因子，而且与暴露在损伤细胞部位的磷脂形成的复合体是否直接激活FXA，从而激活凝血级联的最终共同途径。
Recombinant FVIIa is being developed for treatment of haemophiliacs with antibodies against FVIII/FIX. rFVIIa was shown to be haemostatically active in haemophilia A and B dogs as well as in 20 haemophilia patients (one haemophilia B and 19 haemophilia A patients). Thirteen patients were treated for life-threatening bleedings and nine at surgery (dose: 60-90 micrograms/kg q 3-4 h). One patient underwent synovectomy in a knee joint under the cover of rFVIIa as the sole coagulation factor without any problems. One patient with FXI deficiency was successfully treated at an orchidectomy. The haemophilia B patient was treated in association with a compartment syndrome (surgical fasciotomy) with a complete haemostasis. He later uneventfully underwent skin grafting. Two CNS bleeds, a severe mouth bleed were treated as well as an extensive nasopharyngeal bleed in a patient with an acquired inhibitor against FVIII. Shortening of the prothrombin time as well as of the APTT was seen. No side-effects were observed. It is speculated whether FVIIa in complex with not only tissue factor but also phospholipids exposed at the site of injured cells directly activates FXa and thereby the final common pathway of the coagulation cascade.