D68.01

Billable

Von Willebrand disease, type 1

HCC Category Mapping

V28HCC 112 — Von Willebrand Disease and Other Coagulation Defects

0.247

V24HCC 48 — Coagulation Defects and Other Specified Hematological Disorders

0.209

ESRDHCC 48 — Coagulation Defects and Other Specified Hematological Disorders

0.000

What This Code Means

The most common type of hereditary bleeding disorder affecting blood clotting and platelet function, usually causing mild to moderate bleeding symptoms.

Coding Tips

•Type 1 is the most frequently encountered form of von Willebrand disease
•Document any bleeding episodes or complications in the medical record

Clinical Significance

Von Willebrand disease type 1 is the most common form, accounting for 70-80% of cases, characterized by a partial quantitative deficiency of von Willebrand factor. It typically presents with mild to moderate mucocutaneous bleeding such as epistaxis, menorrhagia, and post-surgical bleeding. Most patients respond well to desmopressin (DDAVP) therapy.

Documentation Requirements

✓Document reduced VWF antigen and VWF activity levels (typically 15-50% of normal), factor VIII levels, and normal multimer distribution.
✓Record bleeding history, DDAVP response test results, and any bleeding episodes requiring intervention.
✓Note family history consistent with autosomal dominant inheritance pattern.

Commonly Confused Codes

D68.00 (Von Willebrand disease, unspecified) — use D68.01 when Type 1 is confirmed D68.020-D68.029 (Type 2 variants) — have qualitative VWF defects with abnormal multimer patterns unlike Type 1 D68.04 (Acquired von Willebrand disease) — similar lab profile but acquired later in life secondary to another condition.

Code Hierarchy

└D68Other coagulation defects └D68.0Von Willebrand disease └D68.01Von Willebrand disease, type 1

└D68.01Von Willebrand disease, type 1