Case 64 – part 2b

Further questioning of our pre-operative patient reveals she has easy bruising without trauma and has required cauterisation for epistaxis in the past. She bled after a tooth was removed and had to see the dentist following the procedure due to bleeding.She uses the pill to control menorrhagia and is iron deficienct as a result. Her basline clotting screen and platelet count are unremarkable. However this does not rule out a bleeding disorder. Looking into family history is important at this stage to give hints towards an inherited condition. Her mother required blood transfusions during pregnancy due to haemorrhage.

 

Inherited bleeding disorders that may have normal coagulation screen include:

  • von Willebrand’s disease
  • Platelet function disorders
  • Alpha-2 anti-plasmin deficiency
  • Factor XIII deficiency
  • Mild factor deficiencies (not severe enough to prolong the APTT)

 

As von Willebrand disease is the most common inherited bleeding disorder first line testing should include a von Willebrand screen:

  • vWF antigen 25 IU/dl  (50-150)
  • vWF ristocetin co-factor 23 IU/dl (50-150)
  • vWF collagen binding assay 22 IU/dl
  • VIII 42 IU/dl (50-150)

 

Questions:

  • What is the diagnosis?
  • What drugs are used to treat this condition?

 

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