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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.

DOI:10.1172/JCI107076
PMID:4626847
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC332957/
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。

相似文献

1
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.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。
J Clin Invest. 1972 Oct;51(10):2593-6. doi: 10.1172/JCI107076.
2
Immunologic studies in von Willebrand's disease. Evidence that the antihemophilic factor (AHF) produced after transfusions lacks an antigen associated with normal AHF and the inactive material produced by patients with classic hemophilia.血管性血友病的免疫学研究。输血后产生的抗血友病因子(AHF)缺乏与正常AHF以及典型血友病患者产生的无活性物质相关的抗原的证据。
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引用本文的文献

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3
Immunologic studies in von Willebrand's disease. Evidence that the antihemophilic factor (AHF) produced after transfusions lacks an antigen associated with normal AHF and the inactive material produced by patients with classic hemophilia.血管性血友病的免疫学研究。输血后产生的抗血友病因子(AHF)缺乏与正常AHF以及典型血友病患者产生的无活性物质相关的抗原的证据。
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4
Interaction of factor VIII antigen in hemophilic plasmas with human antibodies to factor VIII.血友病血浆中因子VIII抗原与人抗因子VIII抗体的相互作用。
J Clin Invest. 1977 May;59(5):984-9. doi: 10.1172/JCI108721.
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Survival of 125iodine-labeled Factor VIII in normals and patients with classic hemophilia. Observations on the heterogeneity of human Factor VIII.125碘标记的凝血因子VIII在正常人和典型血友病患者体内的存活情况。关于人凝血因子VIII异质性的观察。
J Clin Invest. 1978 Aug;62(2):223-34. doi: 10.1172/JCI109120.

本文引用的文献

1
THE FATE OF PROTHROMBIN AND FACTORS VIII, IX AND X TRANSFUSED TO PATIENTS DEFICIENT IN THESE FACTORS.凝血酶原以及凝血因子VIII、IX和X输注给缺乏这些因子的患者后的转归
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2
The survival of factor VIII (antihemophilic globulin) and factor IX (plasma thromboplastin component) in normal humans.正常人中凝血因子VIII(抗血友病球蛋白)和凝血因子IX(血浆凝血活酶成分)的存活情况。
J Clin Invest. 1963 Jul;42(7):1040-7. doi: 10.1172/JCI104789.
3
Production of high-potency concentrates of antihemophilic globulin in a closed-bag system.在密闭袋系统中生产高效抗血友病球蛋白浓缩物。
N Engl J Med. 1965 Dec 30;273(27):1443-7. doi: 10.1056/NEJM196512302732701.
4
Factor 8 detection by hemagglutination inhibition: hemophilia A and von Willebrand's disease.通过血凝抑制检测因子VIII:甲型血友病和血管性血友病。
Science. 1971 Jan 15;171(3967):196-7. doi: 10.1126/science.171.3967.196.
5
Immunologic studies in von Willebrand's disease. Evidence that the antihemophilic factor (AHF) produced after transfusions lacks an antigen associated with normal AHF and the inactive material produced by patients with classic hemophilia.血管性血友病的免疫学研究。输血后产生的抗血友病因子(AHF)缺乏与正常AHF以及典型血友病患者产生的无活性物质相关的抗原的证据。
J Clin Invest. 1972 Oct;51(10):2597-601. doi: 10.1172/JCI107077.