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ICD-10 Coding for Mastocytosis(D47.02, C96.21, D47.01, Q82.2)

Complete ICD-10-CM coding and documentation guide for Mastocytosis. Includes clinical validation requirements, documentation requirements, and coding pitfalls.

Also known as:

Mast cell diseaseSystemic mastocytosisCutaneous mastocytosis

Related ICD-10 Code Ranges

Complete code families applicable to Mastocytosis

D47.0-D47.9Primary Range

Other neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue

This range includes codes for systemic and cutaneous mastocytosis, which are primary conditions of interest.

Malignant mast cell neoplasms

This range includes codes for aggressive systemic mastocytosis, which is a more severe form of the condition.

Congenital cutaneous mastocytosis

This code is used for congenital forms of cutaneous mastocytosis, indicating onset at birth.

Code Comparison: When to Use Each Code

Compare key differences between these codes to ensure accurate selection

CodeDescriptionWhen to UseKey Documentation
D47.02Systemic mastocytosisUse when systemic involvement is confirmed with bone marrow biopsy and elevated tryptase levels.
  • Bone marrow biopsy showing ≥15 mast cell aggregates
  • KIT D816V mutation
  • Serum tryptase >20 ng/mL
C96.21Aggressive systemic mastocytosisUse when aggressive systemic involvement with organ damage is documented.
  • Organ damage such as cytopenias or hepatomegaly
  • C-findings like ascites or hypoalbuminemia
D47.01Cutaneous mastocytosisUse when mastocytosis is limited to the skin without systemic involvement.
  • Positive Darier’s sign
  • Skin biopsy with spindle-shaped mast cells
Q82.2Congenital cutaneous mastocytosisUse when mastocytosis is present from birth.
  • Documentation of congenital origin
  • Presence since birth

Clinical Decision Support

Always review the patient's clinical documentation thoroughly. When in doubt, choose the more specific code and ensure documentation supports it.

Key Information: ICD-10 code for systemic mastocytosis

Essential facts and insights about Mastocytosis

The ICD-10 code for systemic mastocytosis is D47.02, used when systemic involvement is confirmed with bone marrow biopsy and elevated tryptase levels.

Primary ICD-10-CM Codes for mastocytosis

Systemic mastocytosis
Billable Code

Decision Criteria

clinical Criteria

  • Presence of multifocal dense mast cell infiltrates in bone marrow

documentation Criteria

  • Detailed biopsy and genetic test results

Applicable To

  • Indolent systemic mastocytosis
  • Smoldering systemic mastocytosis

Excludes

  • Aggressive systemic mastocytosis (C96.21)
  • Mast cell activation syndrome (D89.8)

Clinical Validation Requirements

  • Bone marrow biopsy showing ≥15 mast cell aggregates
  • KIT D816V mutation
  • Serum tryptase >20 ng/mL

Code-Specific Risks

  • Confusion with mast cell activation syndrome
  • Inadequate documentation of systemic involvement

Coding Notes

  • Ensure documentation specifies systemic involvement and genetic markers.

Ancillary Codes

Additional codes that should be used in conjunction with the main diagnosis codes when applicable.

Fever, unspecified

R50.9
Use to document fever associated with systemic mastocytosis.

Malabsorption, unspecified

K90.0
Use for gastrointestinal complications due to mast cell infiltration.

Differential Codes

Alternative codes to consider when ruling out similar conditions to the primary diagnosis.

Other specified disorders involving the immune mechanism, not elsewhere classified

D89.8
Used for mast cell activation syndrome without systemic mastocytosis criteria.

Systemic mastocytosis

D47.02
Lacks aggressive organ damage or C-findings.

Congenital cutaneous mastocytosis

Q82.2
Onset at birth or infancy.

Cutaneous mastocytosis

D47.01
Acquired after birth.

Documentation & Coding Risks

Avoid these common documentation and coding issues when documenting Mastocytosis to ensure proper reimbursement, maintain compliance, and reduce audit risk. These guidelines are particularly important when using ICD-10 code D47.02.

Impact

Clinical: May lead to misdiagnosis or inappropriate treatment., Regulatory: Non-compliance with coding standards., Financial: Potential loss of reimbursement.

Mitigation Strategy

Ensure detailed biopsy and laboratory findings are documented., Use specific terminology for systemic involvement.

Impact

Reimbursement: Incorrect coding may lead to improper DRG assignment., Compliance: May result in non-compliance with coding standards., Data Quality: Affects accuracy of patient records and data analysis.

Mitigation Strategy

Ensure documentation meets systemic mastocytosis criteria before coding.

Impact

Reimbursement: Incorrect code may affect reimbursement rates., Compliance: Non-compliance with ICD-10 coding guidelines., Data Quality: Leads to inaccurate patient data.

Mitigation Strategy

Confirm and document the onset of the condition.

Impact

Lack of detailed biopsy and laboratory findings.

Mitigation Strategy

Ensure comprehensive documentation of all diagnostic criteria.

Documentation errors, coding pitfalls, and audit risks are interconnected aspects of medical coding and billing. Addressing all three areas helps ensure accurate coding, optimal reimbursement, and regulatory compliance.

Frequently Asked Questions

Common questions about ICD-10 coding for Mastocytosis, with expert answers to help guide accurate code selection and documentation.

Documentation Templates for Mastocytosis

Use these documentation templates to ensure complete and accurate documentation for Mastocytosis. These templates include all required elements for proper coding and billing.

Systemic mastocytosis with bone marrow involvement

Specialty: Hematology

Required Elements

  • Bone marrow biopsy results
  • Serum tryptase levels
  • KIT mutation status

Example Documentation

Systemic mastocytosis (D47.02) confirmed with bone marrow biopsy showing 18 mast cell aggregates, KIT D816V mutation positive, serum tryptase 32 ng/mL.

Examples: Poor vs. Good Documentation

Poor Documentation Example
History of mastocytosis.
Good Documentation Example
Multifocal dense mast cell infiltrates (≥15 cells/aggregate) in bone marrow, CD25+, KIT D816V mutation detected.
Explanation
The good example provides specific biopsy and genetic findings, supporting the diagnosis.

Need help with ICD-10 coding for Mastocytosis? Ask your questions below.

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