von Willebrand Disease Clinical Presentation
- Author: Eleanor S Pollak, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP more...
The most common signs of von Willebrand disease (vWD) include nosebleeds and hematomas. Prolonged bleeding from trivial wounds, oral cavity bleeding, and excessive menstrual bleeding are common. Gastrointestinal bleeding rarely occurs. Other manifestations include the following[8, 9] :
- Easy bruising - Common but nonspecific
- Prolonged bleeding - After minor trauma to skin or mucous membranes
- Severe hemorrhage - After major surgery; less common
- Delayed bleeding - May occur up to several weeks after surgery
- Heavy bleeding - Common after tooth extraction or other oral surgery, such as tonsillectomy and adenoidectomy
- Menorrhagia - Common presenting complaint in women
- Exacerbation of bleeding symptoms - After ingestion of aspirin
- Amelioration of bleeding symptoms with use of oral contraceptives
Pediatric-specific bleeding that may occur in children with vWD include the following :
- Umbilical stump bleeding
- Cheek hematoma
- Conjunctival bleeding
- Post-circumcision bleeding
- Post-venipuncture bleeding
Patients with possible vWD should be asked about any family or personal history of bleeding problems. Any use of medications that might affect coagulation should also be elicited.
Physical examination findings are usually normal. However, patients may show physical sequelae, such as bleeding or bruises. The examiner should note the size, location, and distribution of any ecchymoses, hematomas, or petechiae, and should assess for evidence of risks of increased bleeding, such as the following :
- Jaundice or spider angiomata
- Joint and skin laxity
Udvardy ML, Szekeres-Csiki K, Hársfalvi J. Novel evaluation method for densitometric curves of von Willebrand factor multimers and a new parameter (M(MW)) to describe the degree of multimersation. Thromb Haemost. 2009 Aug. 102(2):412-7. [Medline].
[Guideline] Laffan MA, Lester W, O'Donnell JS, Will A, Tait RC, Goodeve A, et al. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Br J Haematol. 2014 Aug 12. [Medline]. [Full Text].
Sutherland MS, Cumming AM, Bowman M, et al. A novel deletion mutation is recurrent in von Willebrand disease types 1 and 3. Blood. 2009 Jul 30. 114(5):1091-8. [Medline].
Stuijver DJ, Piantanida E, van Zaane B, Galli L, Romualdi E, Tanda ML, et al. Acquired von Willebrand syndrome in patients with overt hypothyroidism: a prospective cohort study. Haemophilia. 2014 May. 20(3):326-32. [Medline].
Waldow HC, Westhoff-Bleck M, Widera C, Templin C, von Depka M. Acquired von Willebrand syndrome in adult patients with congenital heart disease. Int J Cardiol. 2014 Aug 1. [Medline].
Byams VR, Kouides PA, Kulkarni R, et al. Surveillance of female patients with inherited bleeding disorders in United States Haemophilia Treatment Centres. Haemophilia. 2011 Jul. 17 Suppl 1:6-13. [Medline].
Sanders YV, Giezenaar MA, Laros-van Gorkom BA, Meijer K, van der Bom JG, Cnossen MH, et al. von Willebrand disease and aging: an evolving phenotype. J Thromb Haemost. 2014 Jul. 12(7):1066-75. [Medline].
[Guideline] Nichols WL, Hultin MB, James AH, et al, and the NHLBI von Willebrand Disease Expert Panel. The Diagnosis, Evaluation, and Management of von Willebrand Disease. Bethesda, Md: National Heart, Lung, and Blood Institute. NIH publication no. 08-5832. December 2007. [Full Text].
Sanders YV, Fijnvandraat K, Boender J, Mauser-Bunschoten EP, van der Bom JG, et al. Bleeding spectrum in children with moderate or severe von Willebrand disease: Relevance of pediatric-specific bleeding. Am J Hematol. 2015 Sep 16. [Medline].
Roberts JC, Flood VH. Laboratory diagnosis of von Willebrand disease. Int J Lab Hematol. 2015 May. 37 Suppl 1:11-7. [Medline].
Hayward CP, Moffat KA, Graf L. Technological advances in diagnostic testing for von Willebrand disease: new approaches and challenges. Int J Lab Hematol. 2014 Jun. 36(3):334-40. [Medline].
Gill JC, Castaman G, Windyga J, Kouides P, Ragni M, Leebeek FW, et al. Hemostatic efficacy, safety, and pharmacokinetics of a recombinant von Willebrand factor in severe von Willebrand disease. Blood. 2015 Oct 22. 126 (17):2038-46. [Medline]. [Full Text].
Franchini M, Targher G, Montagnana M, Lippi G. Antithrombotic prophylaxis in patients with von Willebrand disease undergoing major surgery: when is it necessary?. J Thromb Thrombolysis. 2009 Aug. 28(2):215-9. [Medline].
Neff AT. Current controversies in the diagnosis and management of von Willebrand disease. Ther Adv Hematol. 2015 Aug. 6 (4):209-16. [Medline].
Di Paola J, Lethagen S, Gill J, et al. Presurgical pharmacokinetic analysis of a von Willebrand factor/factor VIII (VWF/FVIII) concentrate in patients with von Willebrand's disease (VWD) has limited value in dosing for surgery. Haemophilia. 2011 Sep. 17(5):752-8. [Medline].