Factor VIII

  1. HARVEY R. GRALNICK, M.D.;
  2. BARRY S. COLLER, M.D.;
  3. N. RAPHAEL SHULMAN, M.D.;
  4. JUDITH C. ANDERSEN, M.D.; and
  5. MARGARET HILGARTNER, M.D.
  1. Bethesda, Maryland

    Abstract

    Hemophilia and von Willebrand disease are two congenital hemorrhagic disorders associated with a deficiency of factor VIII activity. Newly developed assays for the factor VIII/von Willebrand factor antigen and for the ristocetin cofactor (von Willebrand factor activity) have enabled investigators to better understand the role of the protein in coagulation and its interaction with platelets. A major area of controversy concerns the structure of the protein. One group thinks the factor VIII/von Willebrand factor consists of a single protein that possesses both procoagulant and von Willebrand factor activity. Others believe it is a complex of one large protein with von Willebrand factor and antigen activity and a small protein possessing procoagulant activity. Despite our greater knowledge of the biochemical abnormalities in these disorders, we still do not know why some patients with hemophilia A develop inhibitors. New goals for treatment of patients with hemophilia include prevention of the crippling arthopathy associated with it, improved psychosocial adjustments, and recognition and care of secondary manifestations of the disease or effects of therapy.

    Article and Author Information

    • ▸An edited transcription of a Combined Clinical Staff Conference at the Clinical Center, Bethesda Maryland, 8 January 1976 by the Hematology Service, Department of Clinical Pathology, Clinical Center, National Institutes of Health, U.S. Department of Health, Education, and Welfare.

    • ▸Authors who wish to cite a section of this conference and specifically indicate its author can use this example for the form of reference:

    • COLLER BS: Role of the von Willebrand factor in platelet function, pp. 603-606 in GRALNICK HR (moderator): Factor VIII. Ann Intern Med 86:598-616, 1977

    • *Chief, Hematology Service, Clinical Pathology Department, Clinical Center.

    • ▸Requests for reprints should be addressed to Harvey R. Gralnick, M.D., Chief; Hematology Service, Clinical Pathology Department, Clinical Center, Building 10, Room 5N-236, National Institutes of Health; Bethesda, MD 20014.

      • Received February 22, 1977.
      • Accepted March 7, 1977.
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