Von Willebrand disease, type 3

This code represents Von Willebrand disease (VWD) type 3, the most severe form of this inherited bleeding disorder. It is characterized by a near-complete absence of Von Willebrand factor (VWF) and a significant deficiency of factor VIII, leading to severe spontaneous bleeding episodes. Patients typically present with mucocutaneous bleeding, joint hemorrhages, and gastrointestinal bleeding.

Use this code for patients definitively diagnosed with Von Willebrand disease type 3 based on laboratory testing showing extremely low or undetectable VWF antigen and VWF activity, along with reduced factor VIII levels. This diagnosis is typically made by a hematologist. Documentation should clearly specify "type 3" to support code assignment.

• Ensure documentation explicitly states "type 3" to differentiate from other VWD types.
• Do not confuse with other types of Von Willebrand disease (e.g., type 1, type 2) which have distinct codes.
• Code any associated bleeding manifestations or complications separately, if applicable and supported by documentation.