Angeborenes und erworbenes von-Willebrand-Syndrom

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

• Literatur

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