Menorrhagia and bleeding disorders in adolescent females

Summary

In women, von Willebrand disease (VWD) is the most common inherited bleeding disorder. Since VWD and other inherited bleeding disorders are autosomal disorders, they affect women and men. Menorrhagia, or heavy menstrual bleeding (HMB), is the most common symptom of women with bleeding disorder experience. Objectively, it is defined as bleeding that lasts for more than seven days or results in the loss of more than 80 ml of blood per menstrual cycle. The prevalence of menorrhagia in a woman with a bleeding disorder ranges from 32 to 100% in patients with VWD, from 5 to 98% in patients with a platelet dysfunction and from 35 to 70% in women with a rare factor deficiency. A detailed history and a careful physical exam are the first steps towards a diagnosis in adolescents, adding a PBAC > 100 increased the sensitivity of the screening tool further to 95%. Laboratory testing should be made at the time of menstrual bleeding in an effort to capture the lowest level of VWF : Ag and FVIII : C. Treatment options for menorrhagia in VWD: antifibrinolytic therapy with tranexamic acid, (2) the non-transfusional agent desmopressin (DDAVP), (3) purified blood products that contain factor VIII and VWF concentrated from plasma and (4) hormonal preparations.

Zusammenfassung

Das von-Willebrand-Syndrom (VWS) ist die häufigste angeborene Blutgerinnungsstörung. Pathologisch verstärkte bzw. verlängerte Monatsblutungen (so genannte Menorrhagie) gehören zu den häufigsten Symptome bei Frauen mit Gerinnungsstörungen. Objektiv gesehen sind diese starken Monatsblutungen so definiert, das der Menstruationszyklus mehr als sieben Tage andauert oder aber der Blutverlust mehr als 80 ml pro Zyklus beträgt. Die Prävalenz der Menorrhagie bei Frauen mit einer Blutgerinnungsstörung reicht von 32 bis 100% bei Patientinnen mit VWS, 5 bis 98% bei Patientinnen mit einer Thrombozytenfunktionsstörung und 35 bis 70% bei Frauen mit seltenen Faktorenmangelzuständen. Eine detaillierte Anamnese und eine sorgfältige körperliche Untersuchung sind die ersten Schritte zur Abklärung einer Menorrhagie, bei Vorliegen eines PBAC-Scores > 100 erhöht sich die Empfindlichkeit des Screening-Tools weiter auf 95%. Labortests sollten zum Zeitpunkt des 2.–3. Tages der Monatsblutung durchgeführt werden, in dem Bemühen, den untersten Normwert der von-Willebrand-assoziierten Laborwerte erfassen zu können. Behandlungs-optionen für Menorrhagie bei einem VWS: (1) antifibrinolytische Therapie mit Tran-examsäure, (2) Desmopressin (DDAVP), (3) virus-inaktivierte Plasmafaktoren, die Faktor VIII und VWF enthalten und (4) Hormon-präparate.

Keywords

Menorrhagia - adolescents - bleeding disorders - endometriosis - miscarriage - von Willebrand’s disease

Schlüsselwörter

Hypermenorrhoe - Jugendliche - Gerinnungsstörung - Endometriose - Fehlgeburt - vonWillebrand-Syndrom

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