• defects in the complement system
  • immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
  • sarcoidosis


  • autoimmune disease (systemic) NOS (M35.9)
  • functional disorders of polymorphonuclear neutrophils (D71)
  • human immunodeficiency virus [HIV] disease (B20)
  1. D82 Immunodeficiency associated with other major defects


    • ataxia telangiectasia [Louis-Bar] (G11.3)
    1. D82.0 Wiskott-Aldrich syndrome

      Inclusion term(s):

      • Immunodeficiency with thrombocytopenia and eczema
    2. D82.1 Di George's syndrome

      Inclusion term(s):

      • Pharyngeal pouch syndrome
      • Thymic alymphoplasia
      • Thymic aplasia or hypoplasia with immunodeficiency
    3. D82.2 Immunodeficiency with short-limbed stature

    4. D82.3 Immunodeficiency following hereditary defective response to Epstein-Barr virus

      Inclusion term(s):

      • X-linked lymphoproliferative disease
    5. D82.4 Hyperimmunoglobulin E [IgE] syndrome

    6. D82.8 Immunodeficiency associated with other specified major defects

    7. D82.9 Immunodeficiency associated with major defect, unspecified

Abbreviations used here:

NEC Not elsewhere classifiable
This abbreviation in the Tabular List represents “other specified”. When a specific code is not available for a condition, the Tabular List includes an NEC entry under a code to identify the code as the “other specified” code.

NOS Not otherwise specified
This abbreviation is the equivalent of unspecified.