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ICD-10 Guide
ICD-10 CodesD89.49

D89.49

Billable

Other mast cell activation disorder

BILLABLE STATUSYes
IMPLEMENTATION DATEOctober 1, 2015
LAST UPDATED09/05/2025

Code Description

ICD-10 D89.49 is a billable code used to indicate a diagnosis of other mast cell activation disorder.

Key Diagnostic Point:

Other mast cell activation disorders encompass a range of conditions characterized by inappropriate activation of mast cells, leading to excessive release of mediators such as histamine, cytokines, and other inflammatory substances. These disorders can manifest with a variety of symptoms, including but not limited to, skin reactions (urticaria), gastrointestinal disturbances, respiratory symptoms, and cardiovascular issues. The clinical presentation can vary widely among patients, making diagnosis challenging. Symptoms may be episodic or chronic, and can be triggered by various factors including allergens, stress, infections, and certain medications. The underlying pathophysiology often involves genetic mutations affecting mast cell function or dysregulation of mast cell activation pathways. Accurate diagnosis typically requires a thorough clinical history, physical examination, and may involve laboratory tests such as serum tryptase levels, skin tests, or bone marrow biopsy to assess mast cell proliferation. Treatment strategies often include antihistamines, corticosteroids, and in some cases, medications that stabilize mast cells or target specific pathways involved in mast cell activation.

Code Complexity Analysis

Complexity Rating: Medium

Medium Complexity

Complexity Factors

  • Variety of symptoms leading to potential misdiagnosis
  • Overlap with other hematologic conditions
  • Need for comprehensive patient history and laboratory tests
  • Variability in treatment protocols

Audit Risk Factors

  • Inconsistent documentation of symptoms
  • Lack of clear diagnostic criteria in patient records
  • Failure to document co-existing conditions
  • Inadequate follow-up documentation

Specialty Focus

Medical Specialties

Allergy and Immunology

Documentation Requirements

Detailed patient history, including triggers and symptomatology, laboratory test results, and treatment responses.

Common Clinical Scenarios

Patients presenting with recurrent anaphylaxis, chronic urticaria, or unexplained gastrointestinal symptoms.

Billing Considerations

Ensure that all symptoms and potential triggers are documented to support the diagnosis.

Hematology

Documentation Requirements

Bone marrow biopsy results, serum tryptase levels, and any genetic testing results.

Common Clinical Scenarios

Patients with unexplained cytopenias or mast cell proliferation.

Billing Considerations

Documentation should clearly differentiate between primary mast cell disorders and secondary conditions.

Coding Guidelines

Inclusion Criteria

Use D89.49 When
  • Follow the official ICD
  • CM coding guidelines, ensuring that the diagnosis is supported by clinical documentation
  • Include any relevant laboratory findings and treatment responses

Exclusion Criteria

Do NOT use D89.49 When
  • Exclude conditions that are specifically listed as not covered under this code

Related ICD-10 Codes

Related CPT Codes

85025CPT Code

Complete blood count (CBC) with differential

Clinical Scenario

Used to evaluate blood cell counts in patients suspected of having mast cell activation disorders.

Documentation Requirements

Document the reason for the CBC and any relevant findings.

Specialty Considerations

Allergy and Immunology specialists should correlate CBC results with clinical symptoms.

ICD-10 Impact

Diagnostic & Documentation Impact

Enhanced Specificity

ICD-10 Improvements

The transition to ICD-10 has allowed for more specific coding of mast cell activation disorders, improving the ability to capture the complexity of these conditions and their management.

ICD-9 vs ICD-10

The transition to ICD-10 has allowed for more specific coding of mast cell activation disorders, improving the ability to capture the complexity of these conditions and their management.

Reimbursement & Billing Impact

The transition to ICD-10 has allowed for more specific coding of mast cell activation disorders, improving the ability to capture the complexity of these conditions and their management.

Resources

Clinical References

  • •
    Mast Cell Activation Syndrome: A Comprehensive Guide

Coding & Billing References

  • •
    Mast Cell Activation Syndrome: A Comprehensive Guide

Frequently Asked Questions

What are the common symptoms of mast cell activation disorders?

Common symptoms include skin reactions like hives, gastrointestinal issues such as nausea or diarrhea, respiratory symptoms like wheezing, and cardiovascular symptoms including hypotension.