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v1.0.0
ICD-10 Guide
ICD-10 CodesD81.1

D81.1

Billable

Severe combined immunodeficiency [SCID] with low T- and B-cell numbers

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

Code Description

ICD-10 D81.1 is a billable code used to indicate a diagnosis of severe combined immunodeficiency [scid] with low t- and b-cell numbers.

Key Diagnostic Point:

Severe combined immunodeficiency (SCID) is a rare genetic disorder characterized by a profound deficiency in both T- and B-lymphocytes, leading to a severely compromised immune system. Individuals with SCID are highly susceptible to infections, including opportunistic pathogens, due to the lack of functional immune responses. The condition is often diagnosed in infancy, typically presenting with recurrent infections, failure to thrive, and chronic diarrhea. Laboratory findings reveal markedly low levels of T-cells and B-cells, which are critical for adaptive immunity. Genetic mutations affecting lymphocyte development, such as those in the IL2RG gene, are commonly implicated. Management of SCID includes protective isolation, prophylactic antibiotics, and definitive treatments such as hematopoietic stem cell transplantation (HSCT). Early diagnosis and intervention are crucial for improving outcomes and survival rates in affected individuals.

Code Complexity Analysis

Complexity Rating: High

High Complexity

Complexity Factors

  • Requires understanding of immunologic terminology and concepts.
  • Differentiation from other immunodeficiencies can be challenging.
  • Documentation must specify low T- and B-cell counts.
  • Genetic testing results may need to be included for accurate coding.

Audit Risk Factors

  • Inadequate documentation of laboratory results.
  • Failure to specify the severity of immunodeficiency.
  • Lack of clarity on associated infections or complications.
  • Misclassification with other types of immunodeficiencies.

Specialty Focus

Medical Specialties

Pediatrics

Documentation Requirements

Detailed history of recurrent infections, growth parameters, and immunologic evaluations.

Common Clinical Scenarios

Infants presenting with failure to thrive and recurrent infections.

Billing Considerations

Documentation must include specific immunologic test results and any genetic testing performed.

Immunology

Documentation Requirements

Comprehensive immunologic profile, including T-cell and B-cell counts, and genetic testing results.

Common Clinical Scenarios

Patients with a family history of immunodeficiency or presenting with unusual infections.

Billing Considerations

Accurate coding requires detailed lab results and clinical correlation with symptoms.

Coding Guidelines

Inclusion Criteria

Use D81.1 When
  • Follow official ICD
  • CM guidelines for coding immunodeficiencies
  • Ensure documentation supports the diagnosis, including laboratory findings and clinical symptoms

Exclusion Criteria

Do NOT use D81.1 When
  • Exclude other conditions that may mimic SCID

Related ICD-10 Codes

Related CPT Codes

38240CPT Code

Bone marrow aspiration

Clinical Scenario

Used for diagnostic evaluation of hematologic conditions in SCID patients.

Documentation Requirements

Document indication for aspiration and findings.

Specialty Considerations

Pediatric hematology may require additional documentation for age-specific considerations.

ICD-10 Impact

Diagnostic & Documentation Impact

Enhanced Specificity

ICD-10 Improvements

The transition to ICD-10 has allowed for more specific coding of immunodeficiencies, improving the accuracy of data collection and reimbursement processes for SCID and related conditions.

ICD-9 vs ICD-10

The transition to ICD-10 has allowed for more specific coding of immunodeficiencies, improving the accuracy of data collection and reimbursement processes for SCID and related conditions.

Reimbursement & Billing Impact

reimbursement processes for SCID and related conditions.

Resources

Clinical References

  • •
    Severe Combined Immunodeficiency (SCID) Foundation

Coding & Billing References

  • •
    Severe Combined Immunodeficiency (SCID) Foundation

Frequently Asked Questions

What are the common symptoms of SCID?

Common symptoms include recurrent infections, failure to thrive, chronic diarrhea, and skin rashes. These symptoms typically present in infancy.