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经典血友病患者对抗血友病因子浓缩物输注反应的研究。通过促凝血和免疫技术测定的抗血友病因子半衰期的差异。

Studies on the response of patients with classic hemophilia to transfusion with concentrates of antihemophilic factor. A difference in the half-life of antihemophilic factor as measured by procoagulant and immunologic techniques.

作者信息

Bennett B, Ratnoff O D

出版信息

J Clin Invest. 1972 Oct;51(10):2593-6. doi: 10.1172/JCI107076.

Abstract

Antihemophilic factor (AHF, factor VIII) levels were measured by a standard coagulation method and by an immunologic technique before and after infusion of AHF concentrates into patients with classic hemophilia. After infusion of AHF concentrates, the half-life of the AHF procoagulant (i.e., clot-promoting) activity varied from 12 to 14 hr, whereas that of the antigen ranged from 24 to 40 hr. The half-life of the antigen was similar in patients with and without circulating anticoagulants to AHF. The data are compatible with the suggestion that the antigen may be carried on a precursor molecule which the patient with hemophilia produces but cannot convert to the functional clot-promoting agent. Other explanations of the observations are, however, recognized.

摘要

采用标准凝血方法和免疫技术,在向典型血友病患者输注抗血友病因子(AHF,因子VIII)浓缩物之前和之后,测定AHF水平。输注AHF浓缩物后,AHF促凝(即促凝块形成)活性的半衰期为12至14小时,而抗原的半衰期为24至40小时。有无AHF循环抗凝剂的患者中,抗原的半衰期相似。这些数据与以下观点相符:抗原可能存在于一种前体分子上,血友病患者能够产生这种前体分子,但无法将其转化为具有功能的促凝剂。然而,这些观察结果也存在其他解释。

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