Angeborene plasmatische Gerinnungsstörungen einschließlich von-Willebrand- Syndrom

  • E. Seifried
  • M. M. Müller
  • W. Miesbach
  • J. Oldenburg
Chapter

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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Copyright information

© Springer-Verlag Berlin Heidelberg 2010

Authors and Affiliations

  • E. Seifried
    • 1
  • M. M. Müller
    • 2
  • W. Miesbach
    • 3
  • J. Oldenburg
    • 4
  1. 1.Institut für Transfusionsmedizin u. Immunhämatologie;Klinikum der Johann Wolfgang Goethe-Universität Frankfurt am Main DRK-Blutspendedienst Baden- Württemberg - Hessen gemeinnützige GmbHFrankfurt/Main
  2. 2.Institut für Transfusionsmedizin u. Immunhämatologie;Klinikum der Johann Wolfgang Goethe-Universität Frankfurt am Main DRK-Blutspendedienst Baden-Württemberg - Hessen gemeinnützige GmbHFrankfurt am Main
  3. 3.Medizinische Klinik III - HämophilieambulanzJohann-Wolfgang-Goethe-Universitätsklinik Frankfurt Haus 31Frankfurt
  4. 4.Institut für Experimentelle Hämatologie und TransfusionsmedizinUniversitätsklinikum BonnBonn

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