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    v1.0.28
    OpenPayerBilling CodesCertain disorders involving the immune mechanismICD-10-CM d82-immunodeficiency-with-major-defects
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    ICD-10-CM Code For Immunodeficiency Associated With Other Major Defects: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group D82 includes primary immunodeficiency disorders that are associated with other major congenital or syndromic defects, such as combined immunodeficiencies with additional organ system anomalies. These ICD-10-CM diagnosis codes are used to document clinical scenarios involving inherited or developmental immune dysfunction alongside significant structural or developmental abnormalities, capturing disease severity and multisystem involvement. Accurate coding within the ICD-10-CM D82 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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    ICD-10-CM D82: Immunodeficiency Associated With Other Major Defects Overview

    This group includes primary immunodeficiency disorders that occur together with major congenital defects or syndromic conditions affecting multiple organ systems. These conditions target the immune system and often involve structural or developmental abnormalities in other organs. Accurate coding is important because it reflects clinical complexity and supports correct reimbursement for services related to multisystem care.

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    When to Use

    Use a code from this group when a patient has a documented primary immunodeficiency that is explicitly associated with other major congenital defects or syndromic conditions. These codes are appropriate when clinical documentation specifies both the immunodeficiency diagnosis and the coexisting significant developmental or structural anomalies.

    ICD-10-CM Codes

    7 rows

    ICD-10-CM CodeDescription
    D82.0Wiskott-Aldrich syndrome
    D82.1Di George's syndrome
    D82.2Immunodeficiency with short-limbed stature
    D82.3Immunodeficiency following hereditary defective response to Epstein-Barr virus
    D82.4Hyperimmunoglobulin E [IgE] syndrome
    D82.8Immunodeficiency associated with other specified major defects
    D82.9Immunodeficiency associated with major defect, unspecified

    DRG Codes

    6 rows

    DRG CodeDescription
    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

    Typical Clinical Scenarios

    • A 2-year-old child with a history of recurrent severe bacterial infections, chronic diarrhea, and failure to thrive is referred for immunologic workup; laboratory testing reveals markedly reduced serum immunoglobulins and hypogammaglobulinemia associated with congenital major anomalies on genetic testing. The child presents for evaluation and management of an underlying primary immunodeficiency associated with other structural or chromosomal defects, and a diagnosis from this group is assigned to capture the immunodeficiency tied to those major defects. Typical codes: D82.0, D82.1
    • A 35-year-old adult with a known chromosomal disorder and multiple congenital anomalies is admitted for sepsis from a pneumonia; records document longstanding combined immunodeficiency contributing to recurrent infections and impaired vaccine responses. Because the immunodeficiency is associated with the patient’s other major congenital defects and is complicating the acute infectious presentation, a code from this group is used in the hospitalization record. Typical codes: D82.2, D82.8
    • A pediatric patient with previously diagnosed thymic aplasia and developmental anomalies presents for routine long-term care and monitoring of chronic immunodeficiency, including ongoing prophylactic antibiotics and immunizations withheld due to immune status. The chronic nature of the immune disorder in the setting of other major defects is documented for outpatient management and care coordination, warranting assignment of an appropriate code from this group. Typical codes: D82.3, D82.9

    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

    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

    D89-OTHER-IMMUNE-MECHANISM-DISORDERS

    ICD-10-CM Code For Other Disorders Involving The Immune Mechanism Not Elsewhere Classified: 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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