Platelets, Endothelial Cells and von Willebrand Factor

In plasma and platelets (PI) from 10 patients (pts) with “severe” recessive von Willebrand’s disease (vWD) factor VIII-related antigen (VIIIR:AG) was unmeasurable in 7 and extremely low in 3 using a very sensitive immunoradiometric assay (IRMA); ristocetin cofactor (VIIIR:RCo) was always unmeasurable and VIM:AG was not detectable on immunofluorescence on endothelial cells (EC) of the vessel wall. In 12 pts with “classical”, dominant vWD characterized by reduced plasma level of VIIIR:AG and VI I IR:RCo, normal values were found in PI and the mobility of PI VIIIR:AG on crossed Immunoelectrophoresis was not different from that of normal controls; VIIIRr AG was normal on EC. In 7 pts showing a faster electrophoretic mobility of plasma VIIIR:AG (variant: vWD), the same abnormality was found in PI. VIIIR:AG concentration was normal both in plasma and in PI when measured by electroimmunodiffusion, whereas IRMA gave lower values and VI I IR:RCo was decreased; VIIIR:AG was normal on EC. These findings show that “severe” vWD is the expression of a marked reduction of factor VIII synthesis fully expressed in EC, PI and plasma. In “classical” vWD the plasma defects are not reflected in the cellular compartments, suggesting a defective transfer from EC and PI to plasma. Patients with “variant” vWD are the expression of a qualitative alteration of the factor VIII molecule, functionally defective both in PI and in plasma.

Objekt anzeigen

• Standort: Deutsche Nationalbibliothek Frankfurt am Main
• Umfang: Online-Ressource
• Sprache: Englisch

Verweise und Beziehungen

• Erschienen in: Platelets, Endothelial Cells and von Willebrand Factor ; 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)

Beteiligte, Orts- und Zeitangaben

• Beteiligte Personen und Organisationen: Ruggeri, Z.M.

Weitere Informationen

• DOI: 10.1055/s-0039-1682473
• URN: urn:nbn:de:101:1-2412190829180.266249788544
• Rechteinformation: Open Access unbekannt; Der Zugriff auf das Objekt ist unbeschränkt möglich.