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    v1.0.28
    OpenPayerBilling CodesCertain disorders involving the immune mechanismICD-10-CM d89-other-immune-mechanism-disorders
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    ICD-10-CM Code For Other Disorders Involving The Immune Mechanism Not Elsewhere Classified: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group D89 includes miscellaneous immune-mediated disorders not classified elsewhere, such as immune deficiencies, dysregulation syndromes, and conditions with abnormal immune responses affecting multiple systems. These ICD-10-CM diagnosis codes are used to document clinical scenarios involving immune mechanism abnormalities, capturing disease severity, organ involvement, or clinical complexity relevant to this group. Accurate coding within the ICD-10-CM D89 group supports proper claim adjudication, complete clinical documentation, or appropriate reimbursement.

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    ICD-10-CM D89: Other Disorders Involving The Immune Mechanism, Not Elsewhere Classified Overview

    This group captures immune-mediated disorders that do not fit into other specific immune disease categories and may affect multiple organ systems. These conditions represent dysregulation or abnormal activity of the immune mechanism with varied clinical presentations. Accurate coding matters for reimbursement because it links detailed clinical documentation to appropriate claim processing and supports severity-driven payment and care management decisions.

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    When to Use

    Use a code from this group when the clinical documentation indicates an immune mechanism disorder that does not meet criteria for a more specific ICD-10-CM immune disorder category. Apply these codes to capture the presence of immune dysfunction, its severity, or multisystem involvement when no other precise immune disorder code is appropriate.

    ICD-10-CM Codes

    24 rows

    ICD-10-CM CodeDescription
    D89.0Polyclonal hypergammaglobulinemia
    D89.1Cryoglobulinemia
    D89.2Hypergammaglobulinemia, unspecified
    D89.3Immune reconstitution syndrome
    D89.40Mast cell activation, unspecified
    D89.41Monoclonal mast cell activation syndrome
    D89.42Idiopathic mast cell activation syndrome
    D89.43Secondary mast cell activation
    D89.44Hereditary alpha tryptasemia
    D89.49Other mast cell activation disorder
    D89.810Acute graft-versus-host disease
    D89.811Chronic graft-versus-host disease
    D89.812Acute on chronic graft-versus-host disease
    D89.813Graft-versus-host disease, unspecified
    D89.82Autoimmune lymphoproliferative syndrome [ALPS]
    D89.831Cytokine release syndrome, grade 1
    D89.832Cytokine release syndrome, grade 2
    D89.833Cytokine release syndrome, grade 3
    D89.834Cytokine release syndrome, grade 4
    D89.835Cytokine release syndrome, grade 5

    Showing 1–20 of 24

    DRG Codes

    18 rows

    DRG CodeDescription
    545Connective Tissue Disorders with MCC
    546Connective Tissue Disorders with CC
    547Connective Tissue Disorders without CC/MCC
    808Major Hematological and Immunological Diagnoses Except Sickle Cell Crisis and Coagulation Disorders with MCC
    809Major Hematological and Immunological Diagnoses Except Sickle Cell Crisis and Coagulation Disorders with CC
    810Major Hematological and Immunological Diagnoses Except Sickle Cell Crisis and Coagulation Disorders without CC/MCC
    814Reticuloendothelial and Immunity Disorders with MCC
    815Reticuloendothelial and Immunity Disorders with CC
    816Reticuloendothelial and Immunity Disorders without CC/MCC
    820Lymphoma and Leukemia with Major O.R. Procedures with MCC
    821Lymphoma and Leukemia with Major O.R. Procedures with CC
    822Lymphoma and Leukemia with Major O.R. Procedures without CC/MCC
    823Lymphoma and Non-Acute Leukemia with Other Procedures with MCC
    824Lymphoma and Non-Acute Leukemia with Other Procedures with CC
    825Lymphoma and Non-Acute Leukemia with Other Procedures without CC/MCC
    840Lymphoma and Non-Acute Leukemia with MCC
    841Lymphoma and Non-Acute Leukemia with CC
    842Lymphoma and Non-Acute Leukemia without CC/MCC

    Typical Clinical Scenarios

    • An adult patient presents to the emergency department with acute onset high fevers, generalized lymphadenopathy, mucocutaneous ulcers, and new thrombocytopenia after starting allopurinol one week prior; clinicians suspect a severe hypersensitivity reaction with systemic immune dysregulation and perform urgent workup and supportive care. A diagnosis from this group is assigned when a drug-induced systemic immune-mediated disorder is identified as the cause of multi-organ inflammation and cytopenias. Typical codes: D89.1, D89.2
    • A middle-aged patient in outpatient rheumatology follow-up has chronic, unexplained hypogammaglobulinemia with recurrent sinopulmonary infections and poor vaccine responses; evaluation excludes primary B-cell malignancy and secondary causes, and immunology documents a humoral immune deficiency requiring periodic immunoglobulin replacement. A code from this group is used to capture the non-classified, other disorders involving immune mechanism underlying the chronic susceptibility to infection. Typical codes: D89.0, D89.9
    • A hospitalized patient with sepsis develops new thrombotic thrombocytopenia and low complement levels consistent with secondary complement-mediated immune dysregulation complicating their critical illness; the team records an immune mechanism disorder as a comorbidity influencing management and prognosis. A code from this group is applied to denote the immune-mediated complication contributing to clinical deterioration. Typical codes: D89.89, D89.82

    Related Codes

    D80-IMMUNODEFICIENCY-WITH-PREDOMINANTLY-ANTIBODY-DEFECTS

    ICD-10-CM Code For Immunodeficiency With Predominantly Antibody Defects: Group Overview

    D81-COMBINED-IMMUNODEFICIENCIES

    ICD-10-CM Code For Combined Immunodeficiencies: Group Overview

    D82-IMMUNODEFICIENCY-WITH-MAJOR-DEFECTS

    ICD-10-CM Code For Immunodeficiency Associated With Other Major Defects: Group Overview

    D83-COMMON-VARIABLE-IMMUNODEFICIENCY

    ICD-10-CM Code For Common Variable Immunodeficiency: Group Overview

    D84-OTHER-IMMUNODEFICIENCIES

    ICD-10-CM Code For Other Immunodeficiencies: Group Overview

    D86-SARCOIDOSIS

    ICD-10-CM Code For Sarcoidosis: Group Overview

    Frequently Asked Questions

    References

    1. 1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
    2. 2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
    3. 3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.
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