Von Willebrand disease: key points from the 2008 National Heart, Lung, and Blood Institute guidelines.
Von Willebrand disease (VWD) is the most common inherited bleeding disorder and may affect as many as one in 100 women. The condition results from a deficiency, dysfunction, or absence of von Willebrand factor (VWF). In women, the most common symptom of VWD is menorrhagia. Of women with menorrhagia, 5-20% have been found to have previously undiagnosed VWD. Besides menorrhagia, women with VWD are more likely to experience other conditions that manifest with abnormal reproductive tract bleeding. The patient with a suspected bleeding disorder should be referred to a hemophilia treatment center or hematologist with expertise in bleeding disorders for definitive diagnosis. After diagnosis, the first choice of therapy for the management of menorrhagia in adolescents or adult females who do not desire child bearing is still hormonal contraceptives. Women who fail hormonal contraceptives, yet desire future child bearing, and women who desire pregnancy are candidates for hemostatic therapy, which is generally reserved for patients with VWF levels less than 50 international units/dL. During pregnancy, VWF levels rise, frequently obviating the need for hemostatic therapy at the time of delivery. Minor procedures can be managed with 1-desamino-8-D-arginine vasopressin, antifibrinolytic medication, or both, but major surgery or childbirth requires replacement with VWF and should be conducted in a center with available hematologists, anesthesiologists, pharmacists, and laboratory support experienced in the management of bleeding disorders.


METHODS
III.
James
Andra H
AH
From Duke University Medical Center, Durham, North Carolina.
Manco-Johnson
Marilyn J
MJ
Yawn
Barbara P
BP
Dietrich
Jennifer E
JE
Nichols
William L
WL
D000293:Adolescent; D000328:Adult; D005260:Female; D006801:Humans; D017410:Practice Guidelines as Topic; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D014842:von Willebrand Diseases / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*
James AH. More than menorrhagia: a review of the obstetric and gynaecological manifestations of bleeding disorders. Haemophilia 2005;11:295–307.
Kirtava A, Drews C, Lally C, Dilley A, Evatt B. Medical, reproductive and psychosocial experiences of women diagnosed with von Willebrand’s disease receiving care in haemophilia treatment centres: a case–control study. Haemophilia 2003;9:292–7.
James A, Matchar DB, Myers ER. Testing for von Willebrand disease in women with menorrhagia: a systematic review. Obstet Gynecol 2004;104:381–8.
Sadler JE, Mannucci PM, Berntorp E, Bochkov N, Boulyjenkov V, Ginsburg D, et al. Impact, diagnosis and treatment of von Willebrand disease. Thromb Haemost 2000;84:160–74.
Rodeghiero F, Castaman G, Dini E. Epidemiological investigation of the prevalence of von Willebrand’s disease. Blood 1987;69:454–9.
Werner EJ, Broxson EH, Tucker EL, Giroux DS, Shults J, Abshire TC. Prevalence of von Willebrand disease in children: a multiethnic study. J Pediatr 1993;123:893–8.
Biron C, Mahieu B, Rochette A, Capdevila X, Castex A, Amiral J, et al. Preoperative screening for von Willebrand disease type 1: low yield and limited ability to predict bleeding. J Lab Clin Med 1999;134:605–9.