D68.03

Billable

Von Willebrand disease, type 3

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

A severe inherited bleeding disorder caused by complete or near-complete absence of von Willebrand factor protein, leading to significant bleeding risk.

Coding Tips

•Type 3 is the most severe form; ensure documentation reflects the severity and absence or near-absence of von Willebrand factor
•Document treatment history including factor replacement therapy or desmopressin use to support the diagnosis

Clinical Significance

Von Willebrand disease type 3 is the most severe form, characterized by virtually complete absence of von Willebrand factor, resulting in very low factor VIII levels (typically <10%). Patients experience severe mucocutaneous bleeding and hemophilia-like joint and muscle bleeds. This rare autosomal recessive form accounts for less than 5% of VWD cases and requires regular prophylactic treatment.

Documentation Requirements

✓Document undetectable or severely reduced VWF antigen and activity levels, markedly low factor VIII, and absent VWF multimers.
✓Record frequency and severity of bleeding episodes including joint bleeds, current VWF/FVIII concentrate prophylaxis regimen, and any history of alloantibody development against VWF.

Commonly Confused Codes

D66 (Hemophilia A) — very low factor VIII in Type 3 VWD mimics severe hemophilia D68.01 (Type 1) — quantitative but partial deficiency unlike the near-complete absence in Type 3 D68.00 (Von Willebrand disease, unspecified) — always specify Type 3 when confirmed given its distinct severity.

Code Hierarchy

└D68Other coagulation defects └D68.0Von Willebrand disease └D68.03Von Willebrand disease, type 3

└D68.03Von Willebrand disease, type 3