Current RMA Instruments

Changes from previous Instruments

SOP Bulletin 179

ICD Coding

• ICD-9-CM Codes: 286.4
• ICD-10-AM Codes: D68.0

Brief description

Von Willebrand disease (vWD) is the most common inherited (genetic) bleeding disorder. Von Willebrand's factor (vWF) is an intermediary agent in blood clotting that stimulates platelet adhesion and aggregation over an injured endothelial cell and also stabilizes factor 8 clotting factor, thereby increasing its activity.  vWF is low in vWD and this causes abnormal (inadequate) clotting of blood and abnormal bleeding. 

Confirming the diagnosis

This diagnosis is complex and is based on personal and famility history of bleeding and labotatory testing.  There is no definitive genetic test and such testing is not required for diagnosis.

The relevant medical specialist is a haematologist.

Additional diagnoses covered by SOP

• Nil

Conditions excluded from SOP

• Haemophilia^#
• Thrombocytopaenia - many causes but immune thrombocytopaenia SOP may apply

^#  non-SOP

Clinical onset

The genetic disorder is present from birth.  The condition is often mild and may remain asymptomatic and not come to medical attention.  It may be diagnosied in asymptomatic cases based on familiy history and laboratory testing.  It presents clinically in the form of abnormal bleeding, commonly after trauma, surgery or childbirth.  There may also be prolonged or recurrent epistaxis (nose bleeds), easy bruising, or bleeding following e.g. dental extraction.

Clinical worsening

Treatment is available to manage the risk of bleeding events, e.g. before a procedure, in the form of the drug desmopressin or blood products (vWF concentrate).  These therapies (mostly the latter) are also used to manage emergency bleeding episodes.  Worsening in the form of a serious bleeding event or death could occur if there was an inability to obtain such treatment.