Angeborenes und erworbenes von-Willebrand-Syndrom

R. Schneppenheim; M. Barthels; U. Budde

doi:10.1055/s-0037-1619668

Hämostaseologie, Table of Contents

Hamostaseologie 2005; 25(04): 367-375
DOI: 10.1055/s-0037-1619668

Original Article

Schattauer GmbH

Angeborenes und erworbenes von-Willebrand-Syndrom

Inborn and acquired von Willebrand disease

R. Schneppenheim

¹Klinik für Pädiatrische Hämatologie und Onkologie, Universitätskrankenhaus Eppendorf, Hamburg

,

M. Barthels

²Abteilung Hämatologie, Hämostaseologie und Onkologie, Medizinische Hochschule Hannover

,

U. Budde

³Labor Prof. Arndt und Partner, Hamburg

› Author Affiliations

Abstract

Zusammenfassung

Das von-Willebrand-Syndrom (VWS) ist bekannt für seine ausgesprochene Heterogenität, die sich in der klinischen Symptomatik wie in der Pathophysiologie ausdrückt. Grundlage der phänotypischen Differenzierung sind quantitative und qualitative Unterschiede des von-Willebrand-Faktors sowie seine multimere Struktur. Es werden die klinisch wichtigsten Daten zur Diagnostik und Therapie des angeborenen und erworbenen VWS dargestellt.

Summary

Von Willebrand disease (VWD) is known for its marked heterogeneity which was already recognized by von Willebrand in 1926. The basis of phenotypic differentiation are quantitative and qualitative or functional differences between the different types and subtypes of VWD. Clinical relevant facts for the practioner on diagnosis and therapy of von Willebrand disease and von Willebrand syndrome are presented.

Schlüsselwörter

Angeborenes von-Willebrand-Syndrom - erworbenes von-Willebrand-Syndrom

Keywords

Von Willebrand disease - von Willebrand syndrom

Full Text

References

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