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Related Experiment Videos

Antihemophilic factor (factor VIII).

O D Ratnoff

    Annals of Internal Medicine
    |March 1, 1978
    PubMed
    Summary
    This summary is machine-generated.

    Classic hemophilia involves a deficiency in antihemophilic factor (Factor VIII), while von Willebrand

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    Area of Science:

    • Hematology
    • Biochemistry
    • Molecular Biology

    Background:

    • Classic hemophilia is a coagulative disorder caused by a defect in antihemophilic factor (Factor VIII).
    • Von Willebrand's disease is characterized by a deficiency of antihemophilic factor, leading to impaired platelet aggregation.
    • Hemophiliac plasma contains a variant of antihemophilic factor with deficient clot-promoting properties.

    Purpose of the Study:

    • To elucidate the structural and functional characteristics of antihemophilic factor (Factor VIII).
    • To differentiate the defects in classic hemophilia and von Willebrand's disease.
    • To investigate the potential for detecting hemophilia carriers through immunological measurements.

    Main Methods:

    • Structural analysis of antihemophilic factor to identify subcomponents.

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  • Assessment of ristocetin-induced platelet aggregation in patients with bleeding disorders.
  • Immunological quantification of antihemophilic factor in hemophiliac plasma and carrier samples.

    • Main Results:

    • Antihemophilic factor appears to consist of two subcomponents: a high molecular weight component with platelet aggregation properties and a low molecular weight component with procoagulant activity.
    • Both subcomponents are present in hemophilic plasma, suggesting a qualitative abnormality in the procoagulant component.
    • Carriers of hemophilia may exhibit disproportionately high levels of immunologically measured antihemophilic factor compared to its procoagulant activity.

    Conclusions:

    • Antihemophilic factor (Factor VIII) has distinct structural subcomponents responsible for different functions.
    • The distinction between hemophilia and von Willebrand's disease relates to the specific deficiency or abnormality of these Factor VIII subcomponents.
    • Immunological assays for antihemophilic factor may aid in identifying carriers of hemophilia.