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Von Willebrand's Disease P016

Last amended 
30 June 2015
Current RMA Instruments:
Reasonable Hypothesis SOP
41 of 2015
Balance of Probabilities SOP
42 of 2015
Changes from Previous Instruments:

SOP Bulletin 179

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

This is a genetic disorder affecting the normal blood clotting intermediary agent, von Willebrand’s factor (vWF) causing an abnormal clotting of blood. The major functions of vWF is to stimulate platelet adhesion and aggregation over an injured endothelial cell and stabilizing factor 8 clotting factor thereby increasing its activity.

Is specific diagnostic evidence required to apply the SOP? - Yes.

This diagnosis requires a specialist opinion from a specialist haematologist together with specific diagnostic blood tests being a clotting time, prothrombin time, activated partial Thromboplastin time (APTT), factor 8 and factor 9 assay, platelet count and plasma von Willebrand factor antigen, activity.

Are there sub-factors that require specific diagnostic information? - No.

The only factors are for clinical worsening from pregnancy and inability to obtain appropriate clinical management. It is difficult to ascertain that there is clinical worsening in an intermittent disorder such as this. See notes below.

Additional diagnoses covered by SOP
  • Nil
Conditions excluded from SOP
  • Haemophilia
  • Thrombocytopaenia
Unconfirmed diagnosis

If, after applying the above information, you are unable to confirm the diagnosis, you should then seek medical officer advice about further investigation.

Notes on clinical worsening

Worsening beyond the normal progression of the disease needs to be evident before a clinical worsening factor can be considered.

It is in the nature of this disease to suffer from episodes of uncontrolled bleeding due to inadequate clotting. As such, suffering an episode due to a military related activity is not an aggravation. The aggravation / permanent clinical worsening of any intermittent condition, requires that the frequency of the episodes be increased, the severity of the episodes be increased, or death occurs.