Acquired von Willebrand disease

This code identifies a bleeding disorder that develops later in life, characterized by a deficiency or dysfunction of von Willebrand factor (VWF). Unlike hereditary forms, acquired von Willebrand disease (AVWS) is not inherited but is associated with underlying conditions such as lymphoproliferative disorders, autoimmune diseases, or certain cardiovascular conditions. Patients typically present with mucocutaneous bleeding, easy bruising, or prolonged bleeding after trauma or surgery.

Use this code when documentation clearly indicates a diagnosis of von Willebrand disease that is acquired rather than congenital. This typically occurs in patients with a new onset of bleeding symptoms and a laboratory diagnosis confirming VWF deficiency or dysfunction in the absence of a family history of VWD. Supporting documentation should include laboratory results showing abnormal VWF levels or activity, and a clinical assessment ruling out hereditary forms.

• Differentiate carefully between acquired and hereditary forms of von Willebrand disease; hereditary forms are classified under D68.01-D68.03.
• Always code the underlying condition causing the acquired von Willebrand disease first, if known and applicable, as this is a manifestation code.
• Ensure documentation specifies "acquired" to justify the use of this particular code.