Immunologic Studies in Patients with Von Willebrand’s Disease

An abnormal electrophoretic mobility of factor VIII related antigen (VIIIRAg) is a well known finding in patients with variants of von Willebrand’s disease (vWd), but it has also been described in patients with typical vWd. We have studied 12 patients with vWd and 5 members of a family with a variant of the disease, characterized by normal factor VIII activity and normal VIIIRAg but low or absent ristocetin cofactor activity. The electrophoretic mobility of VIIIRAg as measured by two dimensional immunelectrophoresis at pH 8.6 was grossly abnormal in all patients with a variant of vWd corresponding to the bleeding tendency. This correlation between clinical symptoms and abnormal electrophoretic mobility of VIIIRAg was not found in those patients with vWd, in whom the mobility of VIIIRAg was also grossly abnormal. Chromatography of cryoprecipitate from patients with the described variant of vWd revealed a delayed elution of VIIIRAg with a characteristic pattern, whereas the patients with typical vWd but abnormal electrophoretic mobility of VIIIRAg had normal elution patterns. It is suggested that an abnormal electrophoretic mobility of VIIIRAg is a common finding also in patients with typical vWd.

Object Details

• Location: Deutsche Nationalbibliothek Frankfurt am Main
• Extent: Online-Ressource
• Language: Englisch

References and Relationships

• Bibliographic citation: Immunologic Studies in Patients with Von Willebrand’s Disease ; volume:38 ; number:05 ; year:1977 ; Konferenz: XIIth Congress of The World Federation of Hemophilia; 1977-06-22; Philadelphia ; Thrombosis and haemostasis ; 38, Heft 05 (1977)

Contributors, Places and Time

• Contributor: Böttcher, D. ; Hasler, K. ; Sutor, A.H.

Further information

• DOI: 10.1055/s-0039-1682595
• URN: urn:nbn:de:101:1-2412190854133.246082525938
• Rights: Open Access unbekannt; Der Zugriff auf das Objekt ist unbeschränkt möglich.
• Last update: 15.08.2025, 7:29 AM CEST