D68.021

HCC Category Mapping

What This Code Means

A bleeding disorder caused by abnormal von Willebrand factor protein (type 2B), which affects the blood's ability to clot properly.

Coding Tips

• •Verify the specific type 2B diagnosis is documented; type 2B has distinct platelet-binding abnormalities that differentiate it from other type 2 variants
• •Document any associated symptoms like mucosal bleeding, easy bruising, or prolonged bleeding times to support medical necessity

Clinical Significance

Von Willebrand disease type 2B features a gain-of-function mutation in VWF that causes increased affinity for platelet glycoprotein Ib, leading to spontaneous VWF-platelet binding, consumption of large multimers, and often mild thrombocytopenia. This unique pathophysiology makes desmopressin contraindicated as it can worsen thrombocytopenia. Patients may be misdiagnosed with immune thrombocytopenic purpura.

Documentation Requirements

• ✓Document enhanced ristocetin-induced platelet aggregation at low-dose ristocetin (characteristic finding), loss of high-molecular-weight multimers on analysis, platelet count trends, and genetic testing confirming Type 2B mutation.
• ✓Record why DDAVP is avoided and the VWF-containing concentrate used for treatment.

Commonly Confused Codes