Hämophilie-A-Patienten und Konduktorinnen für Hämophilie A

Summary

The retrospective cohort study surveys the influence of age, co-morbidity and laboratory values on FVIII-activity (FVIII : C) in patients with haemophilia A with (mild n = 48, moderate n = 10, severe n = 7 and carriers n = 23). Median observation was 19 years for patients with haemophilia A and 9,5 years for carriers.

Results FVIII : C levels collected from patients with mild haemophilia A displayed a significant median increase of 6.5% with proceeding age (p = 0.0013). Patients with moderate haemophilia A (and carriers of haemophilia A) showed a non significant median increase of 1.05% (carriers 8%). Eight patients showed FVIII : C levels at last blood withdrawal that indicated a change of severity from moderate to mild haemophilia A. A significant correlation was found between FVIII : C and VWF : RCo (p= 0.0203) and AFP (p < 0.0005). The correlation between FVIII : C and triglycerides and LDH was significant negative (p < 0.0005). No significant correlation could be found for FVIII : C and co-morbidity, fibrinogen, cholesterol and VWF : Ag.

Zusammenfassung

Die retrospektive Kohortenstudie untersucht den Einfluss von Lebensalter, Komorbidität, und Laborparametern auf die Aktivität des Faktor VIII (FVIII : C) bei Patienten mit Hämophilie A (mild n = 48, mittelschwer n = 10, schwer n = 7 und Konduktorinnen für Hämophilie A n = 23). Der mittlere Beobachtungsdauer betrug 19 Jahre für Hämophilie-A-Patienten und 9,5 Jahre für Konduktorinnen für Hämophilie A.

Ergebnisse Es zeigte sich ein signifikanter medianer Anstieg von FVIII : C mit dem Alter von 6,5% bei Patienten mit milder Hämophilie A (p = 0,0013). Patienten mit mittelschwerer Hämophilie A und Konduktorinnen wiesen einen nicht signifikant medianen Anstieg von 1,05% auf (Konduktorinnen 8%) auf.Acht Patienten zeigten bei der letzten Untersuchung FVIII : C einen Wechsel des Schweregrades zur milden Ausprägung. Es korrelierten signifikant positiv FVIII : C und VWF : Rco (p = 0,0203), FVIII : C und AFP (p < 0,0005), signifikant negativ FVIII : C, Triglyceride, FVIII : C und LDH (p < 0,0005). Keine signifikanten Korrelationen zeigten sich zwischen FVIII : C und Komorbidität, Fibrinogen, Cholesterin und VWF : Ag.

Keywords

Haemophilia A - age - FVIII : C - variability

Schlüsselwörter

HämophilieA - Lebensalter - FVIII : C - Variabilität

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