Von Willebrand Disease and other Disorders of Hemostasis in the Patient with Menorrhagia

Abstract

Separately, von Willebrand disease and menorrhagia are two relatively common conditions; in combination they occur at a prevalence of approximately 11–16%. Such patients exhibit a reduced quality of life and can incur a relatively high rate of gynecologic interventions; for example dilatation and curettage, endometrial ablation and hysterectomy. Initial evaluation involves a focused history for the following bleeding symptoms: menorrhagia since menarche, easy bruising of greater than 5 cm 1–2 times/month, frequent gum bleeding when flossing or brushing teeth or epistaxis 1–2 times/month. In addition, for those who have already undergone invasive interventions with the subsequent risk for hemorrhage, inquiry should be made regarding excessive bleeding with childbirth, dental tooth extraction and/or surgery. Step-wise testing includes a complete blood cell count and an assessment of the prothrombin time, activated partial thromboplastin time, iron profile, serum creatinine and thyroid-stimulating hormone level, followed by Factor VIII level, von Willebrand factor antigen and ristocetin cofactor, followed by consideration of platelet aggregation studies. Additional hemostatic studies may include obtaining a Factor XI level and euglobulin clot lysis time. Intuitively, failure to diagnose an underlying hemostatic disorder may lead to continued menorrhagia and diminished quality of life, as well as unnecessary surgical interventions that may in turn be fraught with an increased risk of bleeding. The management of von Willebrand disease-related menorrhagia involves consideration of the patient's age, childbearing status and preference. In the adolescent, surgical intervention is not an option, whereas an older patient beyond her childbearing years may choose a hysterectomy as a definitive treatment in lieu of continued medical therapy with intranasal/subcutaneous 1-deamino-8-D-arginine vasopressin (DDAVP), oral antifibrinolytic agents or oral contraceptive. The sexually active patient may initially choose a trial of oral contraceptive or the levonorgestrel intrauterine device, Mirena®. Pending ongoing comparative trials in von Willebrand disease-related menorrhagia of intranasal DDAVP, tranexamic acid and the levonorgestrel intrauterine device, specific recommendations cannot be made at present regarding the superiority of one intervention compared with another. It should also be noted that the dose and schedule of intranasal DDAVP, tranexamic acid and ε-amino caproic acid have not been well established and warrant further study in combination and at various doses and schedules.