D68.03 Von Willebrand disease, type 3

Billable ICD-10-CM code, FY2026. Acts as a Complication or Comorbidity (CC) as a secondary diagnosis.

Applicable to

• (Near) complete absence of von Willebrand factor
• Total quantitative deficiency of von Willebrand factor

Applies from D68 Other coagulation defects

Excludes1 (never code together)

• abnormal coagulation profile NOS (R79.1)

Excludes2 (not included here)

• coagulation defects complicating abortion or ectopic or molar pregnancy (O00-O07, O08.1)
• coagulation defects complicating pregnancy, childbirth and the puerperium (O45.0, O46.0, O67.0, O72.3)

Applies from D68.0 Von Willebrand disease

Excludes1 (never code together)

• capillary fragility (hereditary) (D69.8)
• factor VIII deficiency NOS (D66)
• factor VIII deficiency with functional defect (D66)

Risk adjustment (HCC)

• CMS-HCC V28: HCC 112 — Immune Thrombocytopenia and Specified Coagulation Defects and Hemorrhagic Conditions
• CMS-HCC V22: HCC 48 — Coagulation Defects and Other Specified Hematological Disorders
• ESRD V24: HCC 48

MS-DRG index

Defines the logic of DRG(s) 813 in MDC 16.

Code annotations containing back-references to D68.03

Back-references to D68.0

• Type 1 Excludes: D66 Hereditary factor VIII deficiency ("factor VIII deficiency with vascular defect (D68.0-)")
• Type 2 Excludes: D69.1 Qualitative platelet defects ("von Willebrand disease (D68.0-)")
• Code First: M36.2 Hemophilic arthropathy ("with vascular defect (D68.0-)")
• Type 1 Excludes: R04.81 Acute idiopathic pulmonary hemorrhage in infants ("von Willebrand disease (D68.0-)")

Back-references to D68

• Type 1 Excludes: R79.1 Abnormal coagulation profile ("coagulation defects (D68.-)")

Diagnosis Index entries for D68.03

• Absence (of) (organ or part) (complete or partial) › von Willebrand factor, complete (near)
• Deficiency, deficient › von Willebrand factor › total quantitative
• Disease, diseased › von Willebrand (-Jürgens) (angiohemophilia) › type 3

Nearby codes

• D68.020 Von Willebrand disease, type 2A
• D68.021 Von Willebrand disease, type 2B
• D68.022 Von Willebrand disease, type 2M
• D68.023 Von Willebrand disease, type 2N
• D68.029 Von Willebrand disease, type 2, unspecified
• D68.03 Von Willebrand disease, type 3 (this code)
• D68.04 Acquired von Willebrand disease
• D68.09 Other von Willebrand disease
• D68.1 Hereditary factor XI deficiency
• D68.2 Hereditary deficiency of other clotting factors
• D68.3 Hemorrhagic disorder due to circulating anticoagulants