Von Willebrand Disease

Von WIllebrand

Discussion of bleeding and bleeding disorders always garners attention. We have recently covered hemophilia, ITP, and hemorrhagic disease of the newborn, but let us turn our attention to the most prevalent bleeding disorder: Von Willebrand Disease.  Certainly, making the diagnosis of von Willebrand disease is not necessarily a common goal in the ED, but knowing how to deal with it is!

 

Von Willebrand Disease Basics

  • Inherited bleeding diathesis.
    • Estimated to affect as much as 1% to 2% of the population (adults and kids).
  • Caused by either too little von Willebrand Factor or von Willebrand factor that is defective.
  • The function of Von Willebrand factor is to help:
    • Bind platelets to injured endothelium
    • Stabilize factor VIII.
  • Von Willebrand is generated in the endothelial cells.

 

Von Willebrand Disease Types

  • TYPE 1
    • Partial Deficiency of von Willebrand Factor
    • Most common type – >70% of cases.
    • Autosomal DOMINANT
    • Usually leads to MILD BLEEDING
  • TYPE 2
    • Defective von Willebrand Factor
    • Has 4 subtypes, depending upon the specific defect.
    • May be autosomal Recessive or Dominant.
    • Severity varies between the subtypes, but more severe than Type 1.
  • TYPE 3
    • Complete Deficiency of von Willebrand Factor.
    • Autosomal RECESSIVE.
    • More Serious presentations.

 

Von Willebrand Disease Presentations

  • Most often presents with mucocutaneous bleeding.
  • Commonly seen:
    • Epistaxis
    • Easy Bruising
    • Menorrhagia
  • Type 3 may present with serious bleeds:
    • Hemarthrosis
    • GI Bleeds

 

Von Willebrand Disease Diagnosis

  • When you are considering a new diagnosis of a bleeding disorder, it is important ask:
    • Age of onset – often presents early, but may be missed until later in life.
    • Location of bleeding
    • Spontaneous bleeding?
    • Family history – obviously… you can’t outrun your genes.
    • Hemostatic Challenges (trauma or surgery) – a major inherited bleeding D/O is unlikely in a patient who has had an uneventful surgery in the past.
  • There are bleeding scoring systems to help determine likelihood of bleeding disorders… but they are not validated in kids.
  • Initial Work-Up
    • CBC
    • Coagulation Studies
    • Von Willebrand Specific Studies
      • vWF Antigen
      • vWF activity
      • Factor VIII level
    • Unfortunately, normal tests do not rule out the diagnosis completely.
    • Involving a hematologist is necessary.

 

Von Willebrand Disease Treatments

  • Desmospressin (DDAVP)
    • Synthetic analog of vasopressin
    • Promotes release of vWF from endothelial cells.
    • Patients with Type 1 often respond well, but type 2 and 3 often do not.
    • Can be given SubQ or IntraNasal, but IV is more effective.
    • Complications = hyponatremia, facial flushing, headache, tachyphlaxis and hypo/hypertension.
  • Plasma-derived vWF and Factor VIII
    • Humate-P
    • Goal is to achieve vWF:RCo > 30% for minor bleeds and >50% for major bleeds.
    • Loading doses:
      • Minor Bleed – 30-50 IU/kg
      • Major Bleed – 40-60 IU/kg
  • Cryoprecipate
    • Contains vWF and FVIII)
      Use only when vWF concentrate is not available.
      1 bag/6 kg body weight.
  • Adjuncts
    • Aminocaproic acid
      • 100-200 mg/kg (max of 10 grams)
      • Followed by 50-100 mg/kg/dose (max of 5 grams) q 6 hrs.
    • Tranexamic acid
      • 25-50 mg/kg q 6-8 hrs.
      • Absorbed from the buccal mucosa and secreted in saliva!
        • Can treat oral bleeding with a mouth-wash of TXA!
        • Crush tablets in 10-15ml of water and have patient hold that in their mouth for 5 min before swallowing.
      • Has longer half-life, more potency, and lower toxicity than Aminocaproic acid and is preferred.

References

Cooper S1, Takemoto C. Von Willebrand disease. Pediatr Rev. 2014 Mar;35(3):136-7; discussion 137. PMID: 24585817. [PubMed] [Read by QxMD]
Bansal D1, Oberoi S, Marwaha RK, Singhi SC. Approach to a child with bleeding in the emergency room. Indian J Pediatr. 2013 May;80(5):411-20. PMID: 23269640. [PubMed] [Read by QxMD]

Singleton T1, Kruse-Jarres R, Leissinger C. Emergency department care for patients with hemophilia and von Willebrand disease. J Emerg Med. 2010 Aug;39(2):158-65. PMID: 18757163. [PubMed] [Read by QxMD]

Sean Fox

I enjoy taking care of patients and I finding it endlessly rewarding to help train others to do the same. I trained at the Combined Emergency Medicine and Pediatrics residency program at University of Maryland, where I had the tremendous fortune of learning from world renown educators and clinicians. Now I have the unbelievable honor of working with an unbelievably gifted group of practitioners at Carolinas Medical Center. I strive every day to inspire my residents as much as they inspire me.

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  1. November 25, 2014

    […] Fox at Pediatric EM Morsels cover the basics of Von Willebrand disease this week. […]

  2. November 25, 2014

    […] More: Von Willebrand Disease (PED EM […]

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