Faktor-XII-Mangel und Thrombophilie

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

• LITERATUR

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