D68.023

HCC Category Mapping

What This Code Means

Von Willebrand disease type 2N is a bleeding disorder where the body produces abnormal von Willebrand factor protein that doesn't work properly, causing difficulty with blood clotting and increased bleeding tendency. This specific type involves a defect in how the von Willebrand factor binds to platelets.

Coding Tips

• •Verify the specific type of von Willebrand disease (type 1, 2A, 2B, 2M, 2N, or 3) is documented in the medical record before assigning this code, as each type has a distinct ICD-10-CM code
• •This code may be used alongside procedure codes for von Willebrand factor testing or treatment codes (such as desmopressin administration) depending on the clinical encounter

Clinical Significance

Von Willebrand disease type 2N (Normandy variant) is characterized by markedly reduced VWF binding affinity for factor VIII, resulting in accelerated factor VIII clearance and low factor VIII levels. This variant mimics mild Hemophilia A and may be misdiagnosed as such. Autosomal recessive inheritance distinguishes it from X-linked Hemophilia A.

Documentation Requirements

• ✓Document VWF:factor VIII binding assay results showing reduced binding capacity, low factor VIII levels with relatively normal VWF antigen and activity, and genetic testing confirming the Type 2N mutation.
• ✓Record family history noting autosomal recessive inheritance pattern and any prior Hemophilia A diagnosis that should be revised.

Commonly Confused Codes