Hamostaseologie 2004; 24(01): 44-49
DOI: 10.1055/s-0037-1619605
Grußwort
Schattauer GmbH

Women with von Willebrand disease

Frauen mit von-Willebrand-Syndrom
I. Scharrer
1   University Hospital, Haemophilia Centre, Frankfurt/Main, Germany
› Author Affiliations
Further Information

Publication History

Publication Date:
22 December 2017 (online)

Summary

The clinical presentation of VWD shows sex-related differences of symptoms. In women the most typical symptoms are menorrhagia, bleeding during and after delivery or abortion and bleeding in connection with caesarean section or gynaecological surgery. Menorrhagia is one of the most common symptoms presented to gynaecologists. In 7-20% of menorrhagia the underlying cause is VWD, in our cohort of 185 women with menorrhagia the prevalence of VWD was even 32%. On the other hand in women with VWD menorrhagia with onset at the menarche can be found in 60-93%, influencing substantially their morbidity and quality of life. During pregnancy women with mild VWD experience a decrease of bleeding tendency due to an increase of endogenous VWF. But as the VWF concentration drops rapidly after delivery, the post-partum period is often associated with significant bleeding complications. In severe forms of VWD the bleeding risk is high during delivery and postpartum period. Laboratory monitoring and therapeutical measures should be continued for 8-10 days after delivery. During menopause the clinical situation improves for most of the women with mild or moderate VWD.

Zusammenfassung

Die klinischen Symptome des VWS unterscheiden sich geschlechtsspezifisch. Typisch für betroffene Frauen sind Menorrhagien, Blutungen während und nach Geburt oder Abort und Blutungskomplikationen bei Schnittentbindung oder gynäkologischen Eingriffen. Die Menorrhagie führt am häufigsten zur Vorstellung beim Gynäkologen. Bei 7-20% liegt der Menorrhagie ein VWS zu Grunde, bei unseren 185 Patientinnen mit Menorrhagie lag die Prävalenz des VWS sogar bei 32%. Andererseits findet man eine Menorrhagie bei 65-93% der Frauen mit VWS, was deren Lebensqualität erheblich beeinflusst. Während der Gravidität stellen Frauen mit mildem VWS eine Abnahme der Blutungstendenz fest, was auf einem Anstieg der VWF-Konzentration beruht. Da diese nach der Entbindung schnell wieder abfällt, treten in der postpartalen Phase häufig schwere Blutungen auf. Bei schweren Formen des VWS ist das Blutungsrisiko während der Geburt und der postpartalen Phase hoch. Die Laborkontrollen und therapeutischen Maßnahmen sollten postpartal für weitere 8-10 Tage fortgesetzt werden. Mit der Menopause verbessern sich die Blutungssymptome der meisten Frauen mit mildem und moderatem VWS.

 
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