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D68.021 ICD-10-CM Code: Von Willebrand disease, type 2B

D68.021 maps to CMS-HCC V28 112. A source-labeled RAF reference is available. Confirm the documented diagnosis and applicable coding requirements. MEAT criteria · RAF Calculator · free HCC coding tools

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Code lookupD68.021

FY 2026 Apr update / Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89) / Coagulation defects, purpura and other hemorrhagic conditions (D65-D69)

D68.021

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Von Willebrand disease, type 2B

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

Buddy the Bee presenting code insight

Buddy Insight

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.

CMS-HCC V28

HCC 112

Coefficient HCC 112: 0.450 (Community Non-Dual Aged (CNA))

CMS-HCC V24

Historical

Historical

Not used for CY2026 payment

ACA/HHS

HCC 075

Code-level coefficient reference

ESRD/PACE

HCC 48

Code-level coefficient reference

RXHCC

N/A

Not mapped

Inclusion Terms

Official
  • Qualitative defects of von Willebrand factor with high-molecular-weight von Willebrand factor loss
  • Qualitative defects of von Willebrand factor with hyper-adhesive forms
  • Qualitative defects of von Willebrand factor with increased affinity for platelet glycoprotein lb
  • Qualitative defects of von Willebrand factorInherited from D68.02

Excludes 2

Official
  • autoimmune disease (systemic) NOS (M35.9)Inherited from D50-D89, D68
  • certain conditions originating in the perinatal period (P00-P96)Inherited from D50-D89, D68
  • complications of pregnancy, childbirth and the puerperium (O00-O9A)Inherited from D50-D89, D68
  • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)Inherited from D50-D89, D68
  • endocrine, nutritional and metabolic diseases (E00-E88)Inherited from D50-D89, D68
  • human immunodeficiency virus [HIV] disease (B20)Inherited from D50-D89, D68
  • injury, poisoning and certain other consequences of external causes (S00-T88)Inherited from D50-D89, D68
  • neoplasms (C00-D49)Inherited from D50-D89, D68
  • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)Inherited from D50-D89, D68
  • coagulation defects complicating abortion or ectopic or molar pregnancy (O00-O07, O08.1)Inherited from D50-D89, D68
  • coagulation defects complicating pregnancy, childbirth and the puerperium (O45.0, O46.0, O67.0, O72.3)Inherited from D50-D89, D68

Includes

Official

No Includes notes are included in this display for D68.021. Check the code and parent instructions in the Code Book.

Excludes 1

Official
  • abnormal coagulation profile NOS (R79.1)Inherited from D68, D68.0
  • capillary fragility (hereditary) (D69.8)Inherited from D68, D68.0
  • factor VIII deficiency NOS (D66)Inherited from D68, D68.0
  • factor VIII deficiency with functional defect (D66)Inherited from D68, D68.0

Code First

Official

No Code First sequencing instructions are included in this display for D68.021. Check the code and parent instructions in the Code Book.

Use Additional

Official

No Use Additional Code instructions are included in this display for D68.021. Check the code and parent instructions in the Code Book.

Code Also

Official

No Code Also instructions are included in this display for D68.021. Check the code and parent instructions in the Code Book.

Buddy Documentation Tip

HCC Buddy guidance
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.

MEAT Support

HCC Buddy guidance
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.

Audit Caution

HCC Buddy guidance
Critical to identify correctly because DDAVP is contraindicated in Type 2B and can precipitate dangerous thrombocytopenia.
Patients may initially be coded as immune thrombocytopenic purpura (D69.3) before the correct diagnosis is established — update the code when Type 2B is confirmed.
Low-dose ristocetin-induced platelet aggregation testing is the key distinguishing lab test.

Common Mistakes

HCC Buddy guidance
D69.3 (Immune thrombocytopenic purpura) — thrombocytopenia in Type 2B may lead to misdiagnosis
D68.020 (Type 2A) — similar multimer loss but different mechanism and different DDAVP response
Platelet-type (pseudo) von Willebrand disease — mutation is in the platelet receptor, not VWF, and has a different code.

Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →

Is D68.021 an HCC code?

Yes. D68.021 (Von Willebrand disease, type 2B) maps to HCC 112, Immune Thrombocytopenia and Specified Coagulation Defects and Hemorrhagic Conditions under the CMS-HCC V28 risk adjustment model, with a source-labeled community non-dual aged reference coefficient of 0.450. Source-labeled code-level coefficients are references, not member totals. Actual contribution depends on complete member context, hierarchy and cleanup rules, interactions, and model year. HCC Buddy's RAF Calculator supports CMS-HCC V28 PY2026 and shows no score unless every required source and calculation check passes. It is billable for payment year 2026.

Coder answer: D68.021 is billable and maps to V28 HCC 112, Immune Thrombocytopenia and Specified Coagulation Defects and Hemorrhagic Conditions. Open it in the Code Book for the tabular path, RAF, and MEAT checklist.

Code
D68.021
Description
Von Willebrand disease, type 2B
HCC (V28)
HCC 112 — Immune Thrombocytopenia and Specified Coagulation Defects and Hemorrhagic Conditions
RAF reference coefficient
0.450
Billable
Yes
Payment year
2026

HCC Category Mapping

V28HCC 112, Immune Thrombocytopenia and Specified Coagulation Defects and Hemorrhagic Conditions
0.450
ESRDHCC 48, Coagulation Defects and Other Specified Hematological Disorders
Not separately weighted

These are source-labeled model coefficients, not member totals. A category may still be removed by hierarchy or model cleanup rules. Weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member context, hierarchy and cleanup rules, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains available for historical review.

Work D68.021 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →

MEAT review for D68.021

For D68.021, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule.

  • MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
  • EEvaluate: test results, medication response, or physical findings reviewed by the provider
  • AAssess: explicit mention in the assessment or plan with acknowledgment of status
  • TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis

Coder workflow notes

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What This Code Means

D68.021 is the ICD-10-CM diagnosis code for von willebrand disease, type 2b. A bleeding disorder caused by abnormal von Willebrand factor protein (type 2B), which affects the blood's ability to clot properly. D68.021 sits in the ICD-10-CM chapter for diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (d50-d89), within the section covering coagulation defects, purpura and other hemorrhagic conditions (d65-d69).

Under the CMS-HCC V28 risk adjustment model, D68.021 maps to Immune Thrombocytopenia and Specified Coagulation Defects and Hemorrhagic Conditions (HCC 112) with a source-labeled community, non-dual, aged reference coefficient of 0.450. No V24 mapping is shown for D68.021; use the applicable model and payment year when reviewing the V28 mapping. For CY2026 non-PACE Medicare Advantage, CMS uses 100% of the 2024 CMS-HCC model (V28). PACE uses a separate model blend. Source-labeled code-level coefficients are references, not member totals. Actual contribution depends on complete member context, hierarchy and cleanup rules, interactions, and model year. HCC Buddy's RAF Calculator supports CMS-HCC V28 PY2026 and shows no score unless every required source and calculation check passes.

Verify the specific type 2B diagnosis is documented; type 2B has distinct platelet-binding abnormalities that differentiate it from other type 2 variants. For D68.021, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.

HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for D68.021 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

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

  • D69.3 (Immune thrombocytopenic purpura): thrombocytopenia in Type 2B may lead to misdiagnosis
  • D68.020 (Type 2A): similar multimer loss but different mechanism and different DDAVP response
  • Platelet-type (pseudo) von Willebrand disease: mutation is in the platelet receptor, not VWF, and has a different code.

Child Codes

Code Hierarchy

D68.021 code history

Code setChange
FY2023 (effective Oct 1, 2022)Added to the code set

Source: official CMS ICD-10-CM order and addenda files, FY2016 through FY2027.

Also searched as

  • D68 021
  • D68021

For D68.021, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule.

D68.021 maps to CMS-HCC V28 category 112, Immune Thrombocytopenia and Specified Coagulation Defects and Hemorrhagic Conditions. See the ICD-10 to HCC mapping hub for how the V28 crosswalk works. The mapping identifies a payment HCC category for D68.021. Review its source-labeled HCC coefficient above, check the RAF Calculator with complete member context for CMS-HCC V28 PY2026, and confirm the documentation the chart needs before the code is submitted. HCC Buddy shows no RAF score unless every required source and calculation check passes.

Work D68.021 in HCC Buddy

Open D68.021 in the Code Book for the full Index-to-Tabular path, MEAT checklist, and V28 HCC mapping, or in the Encoder to code from a keyword search. Pro includes 7 days to try everything, no card required.