von Willebrand Disease Clinical Presentation

Updated: Jun 23, 2017
  • Author: Eleanor S Pollak, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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Presentation

History

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 [9, 10] :

  • 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 [11] :

  • Umbilical stump bleeding
  • Cephalohematoma
  • 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. [9]

Bleeding assessment tools

Bleeding assessment tools (BATs) have been developed to allow clinicians to objectively assess bleeding symptoms in patients with vWD. BAT scores may also help predict bleeding risk in patients with vWD. [12, 13] The International Society on Thrombosis and Haemostasis (ISTH) developed and endorsed a single BAT (ISTH-BAT), to standardize reporting of bleeding symptoms for use in adult and pediatric populations. [14, 12] BATs should always be complemented by coagulation screening tests. [12]

A practical disadvantage of existing BATs is that they must be administered by a physician or other properly trained health professional. Consequently, Deforest and colleagues have developed and validated a self-administered BAT for vWD. Their Self-BAT generated bleeding scores comparable BS to those of ISTH-BAT and proved reliable and effective screening tool for assessment of a possible bleeding disorder, particularly in women. [15]

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Physical Examination

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 [9] :

  • Jaundice or spider angiomata

  • Splenomegaly

  • Arthropathy

  • Joint and skin laxity

  • Telangiectasia

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