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Transfusionsmedizin pp 361–381Cite as

Angeborene plasmatische Gerinnungsstörungen einschließlich Von-Willebrand-Syndrom

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Zusammenfassung

Die häufigsten angeborenen hämorrhagischen Diathesen sind Störungen der plasmatischen Gerinnung. Ursachen sind

  • die quantitative Verminderung eines oder mehrerer Gerinnungsfaktoren

  • verminderte oder fehlende Aktivierbarkeit eines oder mehrerer Gerinnungsfaktoren aufgrund eines qualitativen Defekts

  • die Präsenz eines Inhibitors, der ihre Aktivierung blockiert.

Die häufigsten Störungen sind die Hämophilie A und B und das Von-Willebrand-Syndrom. Selten sind vererbte Mangelzustände der Gerinnungsfaktoren I, II, V, VII, X, XI, XII und XIII. Eine kausale Therapie ist bisher beim Menschen nicht möglich. Das quantitative Ausmaß der Defekte bestimmt die Blutungsneigung, wobei die hämostatische Mindestaktivität für jeden einzelnen Gerinnungsfaktor unterschiedlich ist.

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Authors
  1. E. Seifried
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  1. Institut für Klinische Immunologie und Transfusionsmedizin der Universität, Langhansstr. 7, D-35385, Gießen, Deutschland

    Christian Mueller-Eckhardt

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Seifried, E. (1996). Angeborene plasmatische Gerinnungsstörungen einschließlich Von-Willebrand-Syndrom. In: Mueller-Eckhardt, C. (eds) Transfusionsmedizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-10599-3_22

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  • DOI: https://doi.org/10.1007/978-3-662-10599-3_22

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