VARIANT VONWILLEBRANDS DISEASE - A STUDY EMPHASIZING CROSSED-IMMUNOELECTROPHORESIS

  • 1 January 1980
    • journal article
    • research article
    • Vol. 43  (1) , 2-5
Abstract
Members of an Illinois [USA] kindred were studied with a battery of hemostatic tests including the template bleeding time, platelet retention by glass beads (PRGB), measurement of activities related to factor VIII, and crossed immunoelectrophoresis (CIEP). Nine family members had a history of excessive bleeding, and all 9 had prolonged bleeding times and increased migration of their factor VIII-related antigen (VIIIR:Ag) on CIEP. Of the other tests performed, the VIII:ristocetin cofactor and the PRGB showed the best correlation with the bleeding time. Three subjects who were not bleeders, but who came from a branch of the family where there were several affected members, also had an abnormal VIIIR:Ag. The pattern of inheritance of the altered VIIIR:Ag in this family was autosomal dominant with full penetrance. The CIEP is a valuable screening test for the detection of variant von Willebrand''s disease and the recognition of silent heterozygotes.