HARVEY R. GRALNICK, M.D.; BARRY S. COLLER, M.D.; N. RAPHAEL SHULMAN, M.D.; JUDITH C. ANDERSEN, M.D.; MARGARET HILGARTNER, M.D.
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.
GRALNICK HR, COLLER BS, SHULMAN NR, ANDERSEN JC, HILGARTNER M. Factor VIII. Ann Intern Med. ;86:598–616. doi: 10.7326/0003-4819-86-5-598
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Published: Ann Intern Med. 1977;86(5):598-616.
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