Zusammenfassung
Die Von-Willebrand-Erkrankurig (VWE) wird durch eine qualitative und/oder quantitative Veränderung des Von-Willebrand-Faktors (VWF) ausgelöst (s. Kap. 25). Von den verschiedenen Formen der VWE kann seit 1982 ein als Pseudo-Von-Willebrand-Syndrom (Weiss et al. 1982) oder „Plättchentyp-VWE“ (pitVWE; Miller u. Castella 1982) bezeichneter Erkrankungstyp abgegrenzt werden. Er ist charakterisiert durch eine verstärkte Bindung des VWF an die Thrombozyten, bedingt durch eine fehlerhafte Expression des VWF-Rezeptors Glykoprotein Ib. Im Gegensatz zur echten VWE liegt hier also ein Defekt der Plättchenmembran und nicht ein Defekt des VWF-Moleküls vor. Eventuell auftretende Veränderungen des plasmatischeh VWF sind ätiologisch nur sekundär.
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Kehrel, B., Clemetson, K.J. (1999). Plättchentyp der Von-Willebrand-Erkrankung. In: Müller-Berghaus, G., Pötzsch, B. (eds) Hämostaseologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-07673-6_13
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