Summary
Two recently developed tests for measurement of factor VIII/von Willebrand factor
(FVIII/vWF), i.e. platetelet agglutination by botrocetin and a kinetic latex antigen
assay, were compared with ristocetin cofactor and electroimmunoassay, in respect with
FVIII/vWF size-distribution. FVIII/vWF was measured in six cases of atypical von Willebrand’s
disease (type II), in gel-filtered fractions of normal cryoprecipitate and in the
course of depolymerization of purified normal FVIII/vWF by disulfide reduction. Small
molecular forms of FVIII/vWF from normal and variant type II plasma, and those derived
by disulfide reduction of high-molecular weight FVIII/vWF, showed remarkably decreased
reactivity in ristocetin-, botrocetin- and latex-assay. We conclude that for botrocetin-induced
platelet agglutination, as well as for agglutination of antibody-coated latex particles,
multiple interactions with repeating subunits of FVIII/vWF are required. As a practical
consequence, the combined measurement of FVIII/vWF by the latex test and electroimmunoassay
provides a simple tool for discriminating between the classical von Willebrand’s disease
and its variants.
Keywords
Factor VIII/von Willebrand factor - Botrocetin - Antibody-coated latex particles