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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 guidance

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.

Buddy the Bee presenting code insight

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

HCC 47

RAF 0.665

ACA/HHS

HCC 74

Varies by metal level

ESRD/PACE

HCC 47

RAF 0.078

RXHCC

HCC 99

RAF 0.943

Code Book Path

Official
D89Other disorders involving the immune mechanism, not elsewhere classified
D89.4Mast cell activation syndrome and related disorders
D89.41Monoclonal mast cell activation syndrome

Inclusion Terms

Official

ICD-10-CM does not list inclusion terms for D89.41 in this effective period.

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for D89.41 in this effective period.

Related Child Codes

Official
D89.40Mast cell activation, unspecified
D89.42Idiopathic mast cell activation syndrome
D89.43Secondary mast cell activation
D89.44Hereditary alpha tryptasemia
D89.49Other mast cell activation disorder

Includes

Official

ICD-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

Official

ICD-10-CM does not list Code First sequencing instructions for D89.41 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for D89.41 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for D89.41 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance
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.

MEAT Support

HCC Buddy guidance
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.

Audit Caution

HCC Buddy guidance
Do not assign this code without documentation of clonal mast cell markers
clonality distinguishes this from idiopathic mast cell activation syndrome (D89.42). Ensure the condition does not meet full criteria for systemic mastocytosis, which requires a different code. This code should not be used interchangeably with the unspecified D89.40 once clonality is confirmed.

Common Mistakes

HCC Buddy guidance
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.

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

V24HCC 47, Disorders of Immunity
0.665
ESRDHCC 47, Disorders of Immunity
0.078
RxHCCHCC 99, Immunodeficiencies
0.943

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.

Child Codes

Code Hierarchy

D89.41 code history

Code setChange
FY2017 (effective Oct 1, 2016)Added to the code set

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

Because D89.41 maps to a payment HCC, the documentation must also satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's risk adjustment score.

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