Females with von Willebrand disease: 72 years as the silent majority

Authors

  • C. A. Lee,

  • C. M. Kessler,

  • D. Varon,

  • U. Martinowitz,

  • M. Heim,

  • P. A. KOUIDES

    1. Mary M. Gooley Hemophilia Center, Inc. and the School of Medicine, University of Rochester, Rochester, NY, USA
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Peter A. Kouides, MD, Rochester General Hospital, 1425 Portland Avenue, Rochester, NY 14621, USA. Tel. +1 (716) 338-4081; Fax +1 (716) 338-2347; email: pkouides@rghnet.edu.

Abstract

Summary. The monthly challenge of menstruation as well as the haemostatic challenge of childbirth post partum renders more females than males symptomatic with von Willebrand disease. Among vWD patients, the obstetrical and gynaecological morbidity is certainly more pronounced in Type 2,3 patients compared to Type 1 patients, but even in the latter group there is a high proportion of menorrhagia with associated anaemia, loss of time from work/school and the use of hysterectomy for ultimate control of bleeding. Despite the well known adage of the “gestational palliation” of vWD, there is a high proportion of postpartum haemorrhage in Type 1 patients also especially after the first 24 h after delivery. This may occur despite normalization of the factor VIIIc level in the third trimester, particularly in Type 2,3 patients. With the increasing availability of intranasal/subcutaneous DDAVP that could be readily administered at home for menorrhagia, there recently has been ongoing efforts internationally to determine the prevalence of vWD in females presenting with menorrhagia with a prevalence of 17% combined from two studies of 180 patients total. Issues remain regarding the optimal dose/schedule of intranasal/subcutaneous DDAVP for menorrhagia and the relative efficacy of antifibrinolytic agents. The proper role of oral contraceptives and danazol also deserves further study in vWd patients with menorrhagia. In sum, a comprehensive care approach in females with vWD is warranted analogous to the successful model of care of male haemophiliacs with the intent to (a) reduce unnecessary surgical interventions for menorrhagia, (b) improve the quality of life during menses and (c) optimize peri-partum management.

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