Faktor-XII-Mangel und Thrombophilie

W-M. Halbmayer; Christine Mannhalter; M. Fischer

doi:10.1055/s-0038-1655230

Hämostaseologie, Table of Contents

Hamostaseologie 1993; 13(04): 157-160
DOI: 10.1055/s-0038-1655230

Übersichtsarbeiten/Review Articles

Schattauer GmbH

Faktor-XII-Mangel und Thrombophilie

W-M. Halbmayer

¹Zentrallaboratorium des Krankenhauses der Stadt Wien-Lainz (Vorstand: Univ.-Prof. Dr. M. Fischer)

,

Christine Mannhalter

²Klinisches Institut für Medizinische und Chemische Labordiagnostik*, Allgemeines Krankenhaus, Wien (Stellvertr. d. Vorst.: Univ.-Prof. Dr. H. Rumpold)

,

M. Fischer

¹Zentrallaboratorium des Krankenhauses der Stadt Wien-Lainz (Vorstand: Univ.-Prof. Dr. M. Fischer)

› Author Affiliations

Abstract

Zusammenfassung

Der F-Xll-Mangel wird autosomal rezessiv vererbt und existiert als quantitativer oder qualitativer, dysfunktioneller Mangel. Milde (heterozygote) F-Xll-Mängel weisen meist eine nur geringfügige oder sogar keine Verlängerung der aPTT auf und können somit leicht übersehen werden. Obwohl schwere (homozygote) F-Xll-Mängel deutlich verlängerte aPTT-Zeiten zeigen, wird in Zusammenhang mit dem F-Xll-Mangel üblicherweise keine Blutungsneigung beobachtet, sondern die Patienten neigen im Gegenteil - wie auch John Hageman - zu thromboembolischen Geschehen. Bei Patienten mit rezidivierenden Venenthrombosen wurde eine F-Xll-Mangel-Inzidenz von 8-10% berichtet, und in Patientengruppen’ mit arteriellen Thromboembolien und/oder Herzinfarkt wurde der F-Xll-Mangel sogar noch häufiger beobachtet.

Schlüsselwörter

Faktor XII - F-Xll-Mangel - Thrombophilie - arterielle und venöse Thromboembolien - Myokardinfarkt

Full Text

References

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