D47.Z2 ICD-10-CM Code: Castleman disease
D47.Z2 maps to CMS-HCC V28 21 (RAF 0.671). Documentation must support MEAT. MEAT criteria · RAF calculator · HCC coding software
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FY 2026 Apr update / Neoplasms (C00-D49) / Neoplasms of uncertain behavior, polycythemia vera and myelodysplastic syndromes (D37-D48)
D47.Z2
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceCastleman disease
An abnormal growth of lymphoid tissue characterized by enlarged lymph nodes and excessive antibody production, with uncertain malignant potential.
Buddy Insight
Castleman disease (also known as angiofollicular lymph node hyperplasia) is a rare lymphoproliferative disorder characterized by abnormal lymph node enlargement with characteristic histopathology.
CMS-HCC V28
MappedHCC 21
RAF 0.671
CMS-HCC V24
MappedHCC 48
RAF 0.192
ACA/HHS
MappedHCC 75
Varies by metal level
ESRD/PACE
MappedHCC 48
RAF 0.063
RXHCC
MappedHCC 21
RAF 0.410
Code Book Path
Inclusion Terms
OfficialICD-10-CM does not list inclusion terms for D47.Z2 in this effective period.
Excludes 2
Official- Kaposi's sarcoma (C46.-)
Related Child Codes
Includes
OfficialICD-10-CM does not list Includes notes for D47.Z2 in this effective period.
Excludes 1
OfficialICD-10-CM does not list Excludes 1 notes for D47.Z2 in this effective period.
Code First
OfficialICD-10-CM does not list Code First sequencing instructions for D47.Z2 in this effective period.
Use Additional
OfficialICD-10-CM does not list Use Additional Code instructions for D47.Z2 in this effective period.
Code Also
Official- , if applicable, human herpesvirus 8 infection (B10.89)
Buddy Documentation Tip
MEAT Support
Audit Caution
Common Mistakes
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 D47.Z2 an HCC code?
Yes. D47.Z2 (Castleman disease) maps to Lymphoma and Other Cancers under the CMS-HCC V28 risk adjustment model (and Coagulation Defects and Other Specified Hematological Disorders under V24), with a community non-dual aged RAF of 0.671. It is billable for payment year 2026.
Coder answer: D47.Z2 is billable and maps to V28 HCC 21, Lymphoma and Other Cancers. Open it in the Code Book for the tabular path, RAF, and MEAT checklist.
- Code
- D47.Z2
- Description
- Castleman disease
- HCC (V28)
- HCC 21 — Lymphoma and Other Cancers
- RAF
- 0.671
- Billable
- Yes
- Payment year
- 2026
HCC Category Mapping
Each model's RAF is its CMS base weight for that model's standard population, so 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 segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.
Work D47.Z2 in the Code Book — tabular path, V28 RAF, and MEAT checklist →
MEAT Criteria for D47.Z2
For D47.Z2 to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.
- 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
Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed D47.Z2 during that encounter, not just copy-forwarded from a problem list.
Coder workflow notes
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What This Code Means
D47.Z2 is the ICD-10-CM diagnosis code for castleman disease. An abnormal growth of lymphoid tissue characterized by enlarged lymph nodes and excessive antibody production, with uncertain malignant potential. D47.Z2 sits in the ICD-10-CM chapter for neoplasms (c00-d49), within the section covering neoplasms of uncertain behavior, polycythemia vera and myelodysplastic syndromes (d37-d48).
Under the CMS-HCC V28 risk adjustment model, D47.Z2 maps to Lymphoma and Other Cancers (HCC 21) with a community, non-dual, aged base RAF weight of 0.671. Under the older CMS-HCC V24 model, D47.Z2 maps to Coagulation Defects and Other Specified Hematological Disorders (HCC 48) with a community, non-dual, aged base RAF weight of 0.192. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.
Ensure pathology confirms Castleman disease diagnosis; distinguish between unicentric and multicentric variants in documentation. Because D47.Z2 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. 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, RAF weights, and MEAT documentation criteria for D47.Z2 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •Ensure pathology confirms Castleman disease diagnosis; distinguish between unicentric and multicentric variants in documentation
- •Document any associated symptoms like fever, anemia, or elevated inflammatory markers
Clinical Significance
Castleman disease (also known as angiofollicular lymph node hyperplasia) is a rare lymphoproliferative disorder characterized by abnormal lymph node enlargement with characteristic histopathology. Unicentric disease involves a single lymph node region and is often curable with surgical resection, while multicentric Castleman disease is a systemic inflammatory condition with significant morbidity driven by interleukin-6 dysregulation.
Documentation Requirements
- ✓Documentation must include tissue biopsy confirming Castleman disease histology (hyaline vascular, plasma cell, or mixed type).
- ✓Specify unicentric versus multicentric disease.
- ✓For multicentric disease, document systemic symptoms (fever, night sweats, weight loss), laboratory findings (elevated interleukin-6, C-reactive protein, anemia), and human herpesvirus 8 status.
- ✓Treatment regimen (siltuximab, rituximab) should be documented.
Excludes 2, Not included here, may code separately
- Kaposi's sarcoma (C46.-)
Code Also
- , if applicable, human herpesvirus 8 infection (B10.89)
Commonly Confused Codes
- •C83-C85 (non-Hodgkin lymphoma) may present similarly with lymphadenopathy but has different histopathology.
- •D47.9 (uncertain behavior of lymphoid/hematopoietic tissue, unspecified) is less specific.
- •R59.1 (generalized enlarged lymph nodes) is a symptom code and should not be used when Castleman disease is diagnosed.
- •D76.3 (other histiocytosis syndromes) involves different cell lineages.