Von Willebrand Disease (VWD)

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Von Willebrand Disease is inherited disease due to deficiency of vWF. It is the commonest bleeding  disorder in the population especially in women. 

Clinical presentation 

  • History of easy bruising,  
  • Menorrhagia 
  • Gum bleeding 
  • Joint bleeding in severe cases 

Investigations 

  • Confirmatory test: VWF level assay. 
  • PT, aPTT, and platelet count are normal except in severe cases.  

Pharmacological Treatment                                        

C: etamsylate (PO) 500mg 8hourly until the bleeding stop 

OR 

C: tranexamic acid (PO) 500mg 8hourly until bleeding is stopped. 

If no response    

S: Desmopresin (IV) 0.3µg/kg IV stat. Max. Dose20µg. 

Note: 

  • Patient unresponsive to DDVAP may be treated with virus-inactivated vWF containing FVIII concentrate. 
  • =Never give Etamsylate or Tranexamic acid to patients bleeding per urethral