D68.04

HCC Category Mapping

What This Code Means

A bleeding disorder caused by von Willebrand factor deficiency that develops later in life due to underlying conditions, rather than being inherited from birth.

Coding Tips

• •Identify and document the underlying cause (such as lymphoproliferative disorders, autoimmune conditions, or medications) when possible
• •Distinguish from hereditary von Willebrand disease by confirming the acquired nature and onset in adulthood or secondary to another condition

Clinical Significance

Acquired von Willebrand disease develops in previously healthy individuals secondary to an underlying condition such as lymphoproliferative disorders, myeloproliferative neoplasms, cardiovascular disease (especially aortic stenosis), autoimmune disorders, or hypothyroidism. Unlike hereditary VWD, it typically presents later in life without family bleeding history. Treatment targets both the bleeding and the underlying cause.

Documentation Requirements

• ✓Document the underlying condition causing acquired VWD, absence of prior personal or family bleeding history, VWF antigen and activity levels, and multimer analysis.
• ✓Record response to treatment including DDAVP, VWF concentrates, IVIG, or treatment of the underlying disorder.
• ✓Note when the acquired nature was confirmed relative to the onset of symptoms.

Commonly Confused Codes