D89.41 ICD-10-CM Code: Monoclonal mast cell activation syndrome
D89.41 is not a CMS-HCC payment code. MEAT criteria · RAF calculator · HCC coding software
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FY 2026 Apr update / Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89) / Certain disorders involving the immune mechanism (D80-D89)
D89.41
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceMonoclonal mast cell activation syndrome
A rare disorder where mast cells (immune cells) become abnormally activated due to a specific genetic mutation, causing allergic-like symptoms without an external trigger.

Buddy Insight
Monoclonal mast cell activation syndrome is characterized by episodes of mast cell mediator release in the setting of clonal mast cell populations that do not meet full criteria for systemic mastocytosis.
CMS-HCC V28
N/A—
Not mapped
CMS-HCC V24
MappedHCC 47
RAF 0.665
ACA/HHS
MappedHCC 74
Varies by metal level
ESRD/PACE
MappedHCC 47
RAF 0.078
RXHCC
MappedHCC 99
RAF 0.943
Code Book Path
Inclusion Terms
OfficialICD-10-CM does not list inclusion terms for D89.41 in this effective period.
Excludes 2
OfficialICD-10-CM does not list Excludes 2 notes for D89.41 in this effective period.
Related Child Codes
Includes
OfficialICD-10-CM does not list Includes notes for D89.41 in this effective period.
Excludes 1
Official- aggressive systemic mastocytosis (C96.21)
- congenital cutaneous mastocytosis (Q82.2)
- (non-congenital) cutaneous mastocytosis (D47.01)
- (indolent) systemic mastocytosis (D47.02)
- malignant mast cell neoplasm (C96.2-)
Code First
OfficialICD-10-CM does not list Code First sequencing instructions for D89.41 in this effective period.
Use Additional
OfficialICD-10-CM does not list Use Additional Code instructions for D89.41 in this effective period.
Code Also
OfficialICD-10-CM does not list Code Also instructions for D89.41 in this effective period.
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 D89.41 an HCC code?
Yes. D89.41 maps to Disorders of Immunity under the V24 model but is not retained in V28.
- Code
- D89.41
- Description
- Monoclonal mast cell activation syndrome
- HCC (V28)
- No CMS-HCC V28 mapping
- RAF
- —
- 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 D89.41 in the Code Book — tabular path, V28 RAF, and MEAT checklist →
MEAT Criteria for D89.41
For D89.41 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 D89.41 during that encounter, not just copy-forwarded from a problem list.
Coder workflow notes
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What This Code Means
D89.41 is the ICD-10-CM diagnosis code for monoclonal mast cell activation syndrome. A rare disorder where mast cells (immune cells) become abnormally activated due to a specific genetic mutation, causing allergic-like symptoms without an external trigger. D89.41 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 certain disorders involving the immune mechanism (d80-d89).
Under the older CMS-HCC V24 model, D89.41 maps to Disorders of Immunity (HCC 47) with a community, non-dual, aged base RAF weight of 0.665. 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.
Requires documentation of confirmed monoclonal mast cell population, typically through bone marrow biopsy. Because D89.41 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 D89.41 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •Requires documentation of confirmed monoclonal mast cell population, typically through bone marrow biopsy
- •Differentiate from idiopathic mast cell activation syndrome (D89.42) which lacks the monoclonal component
Clinical Significance
Monoclonal mast cell activation syndrome is characterized by episodes of mast cell mediator release in the setting of clonal mast cell populations that do not meet full criteria for systemic mastocytosis. Patients have evidence of clonality (KIT D816V mutation or aberrant CD25 expression on mast cells) but lack sufficient bone marrow infiltration for a mastocytosis diagnosis. This distinction is clinically important as it may influence monitoring and long-term prognosis.
Documentation Requirements
- ✓Documentation must establish the presence of clonal mast cell markers (KIT D816V mutation, aberrant CD25 or CD2 expression on bone marrow mast cells) alongside clinical evidence of mast cell activation (elevated tryptase during episodes, response to anti-mediator therapy).
- ✓Bone marrow biopsy results excluding systemic mastocytosis criteria should be documented.
- ✓Treatment plan and episode frequency must be recorded.
Commonly Confused Codes
- •D47.02 (Systemic mastocytosis) involves more extensive bone marrow infiltration and meets full WHO criteria.
- •D89.42 (Idiopathic mast cell activation syndrome) lacks clonal markers.
- •D89.40 (Mast cell activation, unspecified) should not be used once monoclonal type is confirmed.
- •C96.20-C96.29 (Mast cell sarcoma/leukemia) represent malignant mast cell neoplasms.

