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Studies on factor VIII activation potential in hemophilia A-plasma and its significance for the comprehension of hemophilia

✍ Scribed by Ann-Marie Mingers

Publisher
Springer
Year
1976
Tongue
English
Weight
629 KB
Volume
123
Category
Article
ISSN
0340-6997

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✦ Synopsis

In a factor VIII exchange test experiment higher factor VIII activities are measurable than would be expected from the single activities of the used plasmas. The same goes for the use of plasmas from carriers of hemophilia A, but not for plasmas from patients with v.W.J.S. (von-Willebrand JΓΌrgens syndrome). In plasmas from hemophilic adults activation obviously proceeds at a slower rate than in plasmas from hemophilic children. The experimental results lead to the hypothesis that factor VIII for its activation needs a specific activator or activator system. This process can be stopped by natural inhibitors. Contrary to existing opinion, the coagulation defect of hemophilia A is not to be sought in an inability to be activated or even in absence of factor VIII, but in a previous step, in its activator. This activator is absent or defective. In patients with v.W.J.S. disease the activator or activator system is intact, but factor VIII is missing.

πŸ“œ SIMILAR VOLUMES

In vitro studies on the way in which the
In vitro studies on the way in which the activation of factor VIII is affected in mixtures of plasma with hemophilia a plasma
✍ Anne-Marie Mingers πŸ“‚ Article πŸ“… 1976 πŸ› Springer 🌐 English βš– 827 KB

Factor VIII exchange test experiments with hemophilia A plasmas were performed to find out how the results were affected by submitting plasmas and plasma mixtures to different incubation periods at 37 degrees C, heat precipitation, and ether extraction. The experiments led to the following results:

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Factor VIII is a multi-domain glycoprotein and is an essential cofactor in the blood coagulation cascade. Its deficiency or dysfunction causes Hemophilia A, a bleeding disorder. Replacement using exogenous recombinant Factor VIII (FVIII) is the first line of therapy for Hemophilia A. Immunogenicity,