2026 ICD-10-CM Diagnosis Code D89.82Autoimmune lymphoproliferative syndrome [ALPS]

ICD-10-CM Codes›D50–D89›D80-D89›D89

ICD-10-CM D89.82
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

D89.82 is a billable ICD-10-CM diagnosis code for autoimmune lymphoproliferative syndrome [ALPS]. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 545 through 547. Coders also document this condition as autoimmune lymphoproliferative syndrome. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Immunity disorders.

Code Identity

ICD-10-CM Code
D89.82
Billable Status
Yes — Valid for Submission
Code Describes
Autoimmune lymphoproliferative syndrome [ALPS]
Short Description
Autoimmune lymphoproliferative syndrome [ALPS]
Same as the full description in the CMS dataset.
Parent Code
D89.8
Other specified disorders involving the immune mechanism, not elsewhere classified

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
CategoryD89Other disorders involving the immune mechanism, not elsewhere classified
This CodeD89.82Autoimmune lymphoproliferative syndrome [ALPS]

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Autoimmune lymphoproliferative syndrome
  • Autoimmune lymphoproliferative syndrome due to CTLA4 haploinsufficiency
  • Autoimmune lymphoproliferative syndrome with recurrent viral infection
  • Dianzani autoimmune lymphoproliferative disease
  • RAS-associated autoimmune leukoproliferative disease
  • STAT3-related early-onset multisystem autoimmune disease

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Autoimmune
      • lymphoproliferative syndrome [ALPS]
    • Syndrome
      • autoimmune lymphoproliferative [ALPS]

Clinical ClassificationClinical

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

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

Clinical InformationClinical

  • Autoimmune Lymphoproliferative Syndrome

    rare congenital lymphoid disorder due to mutations in certain fas-fas ligand pathway genes. known causes include mutations in fas, tnfsf6, nras, casp8, and casp10 proteins. clinical features include lymphadenopathy; splenomegaly; and autoimmunity.

Patient EducationClinical

Autoimmune Diseases

Your immune system protects you from disease and infection by attacking germs that get into your body, such as viruses and bacteria. Your immune system can tell that the germs aren't part of you, so it destroys them. If you have an autoimmune disease, your immune system attacks the healthy cells of your organs and tissues by mistake.

The full article covers:

  • What are autoimmune diseases?
  • What causes autoimmune diseases?
  • Who is at risk for autoimmune diseases?
  • What are the symptoms of autoimmune diseases?
  • How are autoimmune diseases diagnosed?
  • What are the treatments for autoimmune diseases?

Read the full article at MedlinePlus

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

Convert D89.82 to ICD-9-CMHistory

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

ICD-9-CM
279.41 Autoimmun lymphprof synd
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About D89.82Overview

Is D89.82 a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report autoimmune lymphoproliferative syndrome [ALPS] on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does D89.82 group to?

When autoimmune lymphoproliferative syndrome [ALPS] is the principal diagnosis on an inpatient stay, it groups to MS-DRG 545, 546, 547, with relative weights from 0.8362 to 2.4817 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of D89.82?

Under the General Equivalence Mappings, autoimmune lymphoproliferative syndrome [ALPS] converts to ICD-9-CM 279.41 (autoimmun lymphprof synd). The mapping is a direct match.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.