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

Faktor-XII-Mangel und Thrombophilie

W-M. Halbmayer
1   Zentrallaboratorium des Krankenhauses der Stadt Wien-Lainz (Vorstand: Univ.-Prof. Dr. M. Fischer)
,
Christine Mannhalter
2   Klinisches Institut für Medizinische und Chemische Labordiagnostik*, Allgemeines Krankenhaus, Wien (Stellvertr. d. Vorst.: Univ.-Prof. Dr. H. Rumpold)
,
M. Fischer
1   Zentrallaboratorium des Krankenhauses der Stadt Wien-Lainz (Vorstand: Univ.-Prof. Dr. M. Fischer)
› Institutsangaben
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Publikationsverlauf

Publikationsdatum:
26. Juni 2018 (online)

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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
 

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