2027 ICD-10-CM Diagnosis Code D84.1Defects in the complement system

ICD-10-CM Codes›D50–D89›D80-D89›D84

ICD-10-CM D84.1
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

D84.1 is a billable ICD-10-CM diagnosis code for defects in the complement system. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Immunity disorders.

For Medicare Advantage risk adjustment, D84.1 maps to CMS-HCC Category 115 (Specified Immunodeficiencies and White Blood Cell Disorders) under the V28 model, adding a risk factor of about 0.565 for a community, non-dual, aged beneficiary in payment year 2027.

Code Identity

ICD-10-CM Code
D84.1
Billable Status
Yes — Valid for Submission
Code Describes
Defects in the complement system
Short Description
Defects in the complement system
Same as the full description in the CMS dataset.
Parent Code
Other immunodeficiencies

Code Classification

ChapterD50–D89Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
SectionD80-D89Certain disorders involving the immune mechanism
CategoryD84Other immunodeficiencies
This CodeD84.1Defects in the complement system

Medicare Risk Adjustment (HCC)Billing

D84.1 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2027.

CMS-HCC V28 Category (Payment Model)
HCC 115— Specified Immunodeficiencies and White Blood Cell Disorders
Payment HCC · PY 2027 one of 19 ICD-10-CM codes in this category
Risk Adjustment Factor (RAF) Weight
+0.565
community, non-dual, aged · ranges 0.302–0.692 across segments
Hierarchy
Superseded by HCC 114
a more severe related category takes the payment when both are reported
Prior Model (CMS-HCC V24)
HCC 23
V24 retired V28 pays 100% of MA risk scores since PY 2026
Other CMS Models
PACE (CMS-HCC V22): HCC 23 · ESRD (V21): HCC 23 · ESRD (V24): HCC 23
ESRD V21 weights: 0.013 dialysis, 0.234–0.359 functioning graft · ESRD V24 weights: 0.036 dialysis, 0.217–0.390 functioning graft
Part D (RxHCC)
RxHCC 98 — Hereditary Angioedema and Other Defects in the Complement System
also risk-adjusts in the Part D prescription drug model (V08)

Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Acquired angioedema due to C1 inhibitor autoantibody
  • Acquired C1 esterase inhibitor deficiency
  • Alternative pathway deficiency
  • Anaphylotoxin inactivator deficiency
  • Anemia due to mechanical damage
  • Angioedema due to disorder of kinin metabolism
  • Angioedema of gingiva due to deficiency of C1 esterase inhibitor
  • Atypical hemolytic uremic syndrome
  • Atypical hemolytic uremic syndrome with anti-factor H antibodies
  • Atypical hemolytic uremic syndrome with complement gene abnormality
  • Autoimmune angioedema
  • Autoimmune urticaria and/or angioedema
  • Classical complement pathway abnormality
  • Combined complement 6 and 7 deficiencies
  • Complement 1q beta chain deficiency
  • Complement 1q deficiency
  • Complement 1q dysfunction
  • Complement 1r deficiency
  • Complement 1s deficiency
  • Complement 2 deficiency
  • Complement 4 binding protein deficiency
  • Complement 4 deficiency
  • Complement 4A deficiency
  • Complement 4B deficiency
  • Complement 5 deficiency
  • Complement 5 dysfunction
  • Complement 5a inhibitor deficiency
  • Complement 6 deficiency
  • Complement 7 deficiency
  • Complement 8 alpha-gamma deficiency
  • Complement 8 beta chain deficiency
  • Complement 8 beta chain dysfunction
  • Complement 9 deficiency
  • Complement abnormality
  • Complement component 3 deficiency
  • Complement component 8 deficiency
  • Complement component deficiency
  • Complement deficiency disease
  • Complement hyperactivation, angiopathic thrombosis, protein losing enteropathy syndrome
  • Complement receptor 1 deficiency
  • Complement receptor 3 deficiency
  • Complement receptor deficiency
  • Complement regulatory factor defect
  • Coombs negative hemolytic anemia
  • Cutis laxa with complement deficiency
  • Cutis laxa, acquired type
  • Decay accelerating factor deficiency
  • Disorder of complement
  • Edema of oral soft tissues
  • Factor B deficiency
  • Factor D deficiency
  • Factor H deficiency
  • Familial C3B inhibitor deficiency syndrome
  • Gingival edema
  • Glomerular disease due to complement system disorder
  • Hereditary angioedema
  • Hereditary angioedema with C1Inh deficiency
  • Hereditary C1 esterase inhibitor deficiency - deficient factor
  • Hereditary C1 esterase inhibitor deficiency - dysfunctional factor
  • Homologous restriction factor deficiency
  • Immunodeficiency due to MASP-2 deficiency
  • Panniculitis due to immunological disorder
  • Panniculitis with complement deficiency
  • Primary CD59 deficiency
  • Properdin deficiency disease
  • Protein-losing enteropathy
  • Recurrent infection caused by Gram-negative bacteria
  • Recurrent infection caused by Neisseria
  • Recurrent Neisseria infection due to factor D deficiency
  • Terminal component deficiency

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to D84.1: its own notes plus those printed at block D80-D89 and Chapter 3. A note printed at a category, block or chapter applies to every code under it.

Includes

Further defines, or gives examples of, the content of the category.

From Block D80-D89 Certain disorders involving the immune mechanism applies to 83 codes
  • defects in the complement system
  • immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
  • sarcoidosis

Applicable To

Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.

  • C1 esterase inhibitor [C1-INH] deficiency

Excludes1

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

From Block D80-D89 Certain disorders involving the immune mechanism applies to 83 codes
  • autoimmune disease (systemic) NOS (M35.9)
  • functional disorders of polymorphonuclear neutrophils (D71-)

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From Block D80-D89 Certain disorders involving the immune mechanism applies to 83 codes
  • human immunodeficiency virus [HIV] disease (B20)
From Chapter 3 (D50-D89) Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism applies to 324 codes
  • autoimmune disease (systemic) NOS (M35.9)
  • certain conditions originating in the perinatal period (P00-P96)
  • complications of pregnancy, childbirth and the puerperium (O00-O9A)
  • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
  • endocrine, nutritional and metabolic diseases (E00-E88)
  • human immunodeficiency virus [HIV] disease (B20)
  • injury, poisoning and certain other consequences of external causes (S00-T88)
  • neoplasms (C00-D49)
  • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name D84.1, its category, or a range that includes it.

Excludes1 3

These codes carry an Excludes1 note naming D84.1: they are not reported together with it, unless the two conditions are unrelated.

  • D84.81 Immunodeficiency due to conditions classified elsewhere
    certain disorders involving the immune mechanism (D80-D83, D84.0, D84.1, D84.9)
  • L50 Urticaria
    hereditary angio-edema (D84.1)
  • D72 Other disorders of white blood cells
    immunity disorders (D80-D89) names D80-D89, which includes this code

Code Also 1

These codes say D84.1 may also be needed to fully describe the condition. The note gives no sequencing direction.

  • D59.32 Hereditary hemolytic-uremic syndrome
    defects in the complement system (D84.1)

Index to Diseases and InjuriesGuidance

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR BLD008
Immunity disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Patient EducationClinical

Immune System and Disorders

Your immune system is a complex network of cells, tissues, and organs. Together they help the body fight infections and other diseases.

The full article covers:

  • What is the immune system?
  • What are the parts of the immune system?
  • How does the immune system work?
  • What are the types of immunity?
  • What can go wrong with the immune system?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert D84.1 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
277.6 Defic circul enzyme NEC
Approximate The match is approximate rather than exact.
ICD-9-CM
279.8 Immune mechanism dis NEC
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About D84.1Overview

What is the ICD-10 code for defects in the complement system?

The ICD-10-CM code for defects in the complement system is D84.1 (sometimes written as D841). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is D84.1 (Other immunodeficiencies) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report defects in the complement system on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does D84.1 group to?

When defects in the complement system is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.

Can D84.1 be reported with M35.9?

Not as a rule. The Excludes1 note at block D80-D89, which applies to D84.1, lists "autoimmune disease (systemic) NOS (M35.9)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).

What is the ICD-9 equivalent of D84.1?

Under the General Equivalence Mappings, defects in the complement system converts to ICD-9-CM 277.6 (defic circul enzyme NEC) and 279.8 (immune mechanism dis NEC). The mapping is approximate, so confirm the match fits the documentation.

What HCC is D84.1?

D84.1 (defects in the complement system) maps to CMS-HCC Category 115 (Specified Immunodeficiencies and White Blood Cell Disorders), commonly written as HCC 115, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2027. It mapped to HCC 23 under the retired V24 model. It also maps in the PACE (CMS-HCC V22), ESRD (V21), and ESRD (V24) models. In the Part D prescription drug model it maps to RxHCC 98.

Does D84.1 risk-adjust for Medicare Advantage payment?

Yes. When documented and reported on a Medicare Advantage encounter, D84.1 adds a risk adjustment factor of about 0.565 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 0.302 to 0.692 depending on the payment segment). A more severe related category (HCC 114) supersedes it when both are reported. See the full factor table on the HCC 115 category page.