D84.1 Defects in the complement system

✓ Billable ICD-10-CM 2026
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The ICD-10-CM code for Defects in the complement system is D84.1 (FY2026). It is a billable, claim-ready diagnosis code.

Classification

Section
D80-D89: Certain disorders involving the immune mechanism (D80-D89)
Category D84
10 codes (7 billable)
FY2026 Status
Stable since FY2024

Also Known As

ICD-10-CM Alphabetic Index entries that lead to D84.1:

  • Defect, defective › complement system
  • Quincke's disease or edema › hereditary
  • Deficiency, deficient › C1 esterase inhibitor (C1-INH)
  • Bannister's disease › hereditary
  • Angioneurotic edema (allergic) (any site) (with urticaria) › hereditary
  • Angioedema (allergic) (any site) (with urticaria) › hereditary
  • Edema, edematous (infectious) (pitting) (toxic) › circumscribed, acute › hereditary
  • Edema, edematous (infectious) (pitting) (toxic) › angioneurotic (allergic) (any site) (with urticaria) › hereditary

Inclusion Terms

  • C1 esterase inhibitor [C1-INH] deficiency

U.S. Hospital Utilization

  • An estimated 2,660 U.S. inpatient stays in 2023 included D84.1 among the documented diagnoses.
  • 775 stays listed it as the principal diagnosis.

Source: National Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality, 2016–2023. National survey-weighted estimates.

Official Coding Guidelines

Chapter 3 TOC entry

3. Chapter 3: Disease of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89) ............................................................................................. 39

— ICD-10-CM Official Guidelines for Coding and Reporting, FY2026, Section I.C.3
Reserved for future guideline expansion

Reserved for future guideline expansion ...................................................................................... 39

— ICD-10-CM Official Guidelines for Coding and Reporting, FY2026, Section I.C.3.Reserved

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

Related Codes

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Reviewed by Prajwal Shrestha, CPC, CRC
Certified Professional Coder (CPC) and Certified Risk Adjustment Coder (CRC) · AAPC Member ID 01997614 · About · Editorial policy · Content last reviewed: 2025-10-01

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