What is ICD-10 code D89.82?

D89.82 is the ICD-10-CM diagnosis code for Autoimmune lymphoproliferative syndrome [ALPS]. A rare inherited immune disorder where the body cannot properly control lymphocyte (immune cell) growth, leading to enlarged lymph nodes, spleen, and increased risk of infections and cancers. D89.82 is a billable code, meaning it can be reported on a claim as a primary or secondary diagnosis when supported by documentation. D89.82 is part of the FY2026 ICD-10-CM code set published by the CMS National Center for Health Statistics and used on Medicare, Medicaid, and commercial claims in the United States.

Yes. D89.82 is a billable ICD-10-CM code that can be reported on professional and institutional claims as a primary or secondary diagnosis when the provider documentation supports the specific condition described. Billable codes in ICD-10-CM are coded to the highest level of specificity available for the documented diagnosis. D89.82 is current for fiscal year 2026 and remains in the active CMS ICD-10-CM code set published by the National Center for Health Statistics.

What is the V28 vs V24 difference for D89.82?

CMS phased in the V28 risk adjustment model over payment years 2024 (33%), 2025 (67%), and 2026 (100%), replacing V24. V28 consolidates HCC categories, removes some lower-acuity conditions, and recalibrates RAF weights across the entire model. D89.82 was a payment HCC under V24 (Disorders of Immunity) but the V28 recalibration removed it from the risk adjustment model. Both models can still appear in legacy data and in payer-specific lookback periods.

What should coders know when reporting D89.82?

ALPS is a genetic disorder; document any family history and genetic testing results in the medical record This is a rare disease; ensure proper documentation to support medical necessity for specialized treatments Because D89.82 maps to a payment HCC, the documentation must also satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's risk adjustment score.

D89.82: Autoimmune lymphoproliferative syndrome

ICD-10-CM Code View

HCC Buddy Code Card

Digital ICD-10 code-book layout with official code detail, always-visible risk models, Code Trumping, and Buddy coding guidance.

Code lookupD89.82Effective periodFY2026 Apr Update (Apr 1-Sep 30)

FY 2026 Apr update / Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89) / Certain disorders involving the immune mechanism (D80-D89)

D89.82

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Autoimmune lymphoproliferative syndrome [ALPS]

A rare inherited immune disorder where the body cannot properly control lymphocyte (immune cell) growth, leading to enlarged lymph nodes, spleen, and increased risk of infections and cancers.

Buddy Insight

CMS-HCC V28

Not risk-adjusted for this model/period.

RAF 0

CMS-HCC V24

Mapped

HCC 47

Disorders of Immunity

RAF 0.472

ACA/HHS

Not risk-adjusted for this model/period.

RAF 0

ESRD/PACE

Mapped

HCC 47

Disorders of Immunity

RAF 0.0

RXHCC

Not risk-adjusted for this model/period.

RAF 0

Code Trumping

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Code Book Path

Official

D89Other disorders involving the immune mechanism, not elsewhere classified

D89.8Other specified disorders involving the immune mechanism, not elsewhere classified

D89.82Autoimmune lymphoproliferative syndrome [ALPS]

Inclusion Terms

Official

ICD-10-CM does not list inclusion terms for D89.82 in this effective period.

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for D89.82 in this effective period.

Related Child Codes

Official

D89.81Graft-versus-host disease

D89.83Cytokine release syndrome

D89.84IgG4-related disease

D89.89Other specified disorders involving the immune mechanism, not elsewhere classified

Includes

Official

ICD-10-CM does not list Includes notes for D89.82 in this effective period.

Excludes 1

Official

ICD-10-CM does not list Excludes 1 notes for D89.82 in this effective period.

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for D89.82 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for D89.82 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for D89.82 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance

Documentation should include the confirmed diagnosis of autoimmune lymphoproliferative syndrome with supporting evidence such as elevated double-negative T cells (CD3+CD4-CD8-), FAS gene mutation analysis, elevated biomarkers (soluble FAS ligand, interleukin-10, vitamin B12), and clinical features.

Current treatment (immunosuppressive agents, sirolimus, mycophenolate), presence of autoimmune cytopenias, and lymphoma surveillance status should be documented.

MEAT Support

HCC Buddy guidance

Current treatment (immunosuppressive agents, sirolimus, mycophenolate), presence of autoimmune cytopenias, and lymphoma surveillance status should be documented.

Audit Caution

HCC Buddy guidance

This is a specific genetic diagnosis

do not assign based on lymphoproliferation alone without documented diagnostic criteria being met. If the patient develops lymphoma as a complication of autoimmune lymphoproliferative syndrome, assign the lymphoma code as the principal diagnosis with autoimmune lymphoproliferative syndrome as a contributing condition. Ensure autoimmune cytopenias are coded separately when present.

Common Mistakes

HCC Buddy guidance

D47.Z1 (Post-transplant lymphoproliferative disorder) involves lymphoproliferation after transplant immunosuppression, not a genetic defect. C85.9

(Non-Hodgkin lymphoma, unspecified) should be coded if malignant transformation has occurred. D89.89 (Other specified disorders involving the immune mechanism) is less specific and should not be used when autoimmune lymphoproliferative syndrome is confirmed. D69.3 (Immune thrombocytopenic purpura) may be an associated finding but does not replace this code.

Last updated: FY2026 ICD-10-CM Apr update, Apr 1, 2026 through Sep 30, 2026. CMS-HCC V28 is 100% phased in for payment year 2026.

Is D89.82 an HCC code?

Yes. D89.82 maps to Disorders of Immunity under the V24 model but is not retained in V28.

HCC Category Mapping

V24HCC 47, Disorders of Immunity

0.472

ESRDHCC 47, Disorders of Immunity

0.000

RAF weights shown are the community, non-dual, aged base weights from the CMS risk adjustment model file. Actual per-patient RAF contribution depends on member segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.

MEAT Criteria for D89.82

For D89.82to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.

MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
EEvaluate: test results, medication response, or physical findings reviewed by the provider
AAssess: explicit mention in the assessment or plan with acknowledgment of status
TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis

Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed D89.82 during that encounter, not just copy-forwarded from a problem list.

What This Code Means

D89.82 is the ICD-10-CM diagnosis code for autoimmune lymphoproliferative syndrome [alps]. A rare inherited immune disorder where the body cannot properly control lymphocyte (immune cell) growth, leading to enlarged lymph nodes, spleen, and increased risk of infections and cancers. D89.82 sits in the ICD-10-CM chapter for diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (d50-d89), within the section covering certain disorders involving the immune mechanism (d80-d89).

Under the older CMS-HCC V24 model, D89.82 maps to Disorders of Immunity (HCC 47) with a community, non-dual, aged base RAF weight of 0.472. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.

ALPS is a genetic disorder; document any family history and genetic testing results in the medical record. Because D89.82 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.

HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for D89.82 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

•ALPS is a genetic disorder; document any family history and genetic testing results in the medical record
•This is a rare disease; ensure proper documentation to support medical necessity for specialized treatments

Clinical Significance

Autoimmune lymphoproliferative syndrome is a rare inherited disorder of lymphocyte apoptosis, typically caused by mutations in the FAS gene, leading to chronic lymphoproliferation, autoimmune cytopenias (particularly autoimmune hemolytic anemia and immune thrombocytopenia), and increased risk of lymphoma. It typically presents in childhood with splenomegaly and lymphadenopathy. Lifelong monitoring for malignant transformation is essential.

Documentation Requirements

✓Documentation should include the confirmed diagnosis of autoimmune lymphoproliferative syndrome with supporting evidence such as elevated double-negative T cells (CD3+CD4-CD8-), FAS gene mutation analysis, elevated biomarkers (soluble FAS ligand, interleukin-10, vitamin B12), and clinical features.
✓Current treatment (immunosuppressive agents, sirolimus, mycophenolate), presence of autoimmune cytopenias, and lymphoma surveillance status should be documented.

Commonly Confused Codes

•D47.Z1 (Post-transplant lymphoproliferative disorder) involves lymphoproliferation after transplant immunosuppression, not a genetic defect. C85.9
•(Non-Hodgkin lymphoma, unspecified) should be coded if malignant transformation has occurred. D89.89 (Other specified disorders involving the immune mechanism) is less specific and should not be used when autoimmune lymphoproliferative syndrome is confirmed. D69.3 (Immune thrombocytopenic purpura) may be an associated finding but does not replace this code.