Includes:

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

Excludes1

  • autoimmune disease (systemic) NOS (M35.9)
  • functional disorders of polymorphonuclear neutrophils (D71)
  • human immunodeficiency virus [HIV] disease (B20)
  1. D81 Combined immunodeficiencies

    Excludes1

    • autosomal recessive agammaglobulinemia (Swiss type) (D80.0)
    1. D81.0 Severe combined immunodeficiency [SCID] with reticular dysgenesis

    2. D81.1 Severe combined immunodeficiency [SCID] with low T- and B-cell numbers

    3. D81.2 Severe combined immunodeficiency [SCID] with low or normal B-cell numbers

    4. D81.3 Adenosine deaminase [ADA] deficiency

      1. D81.30 Adenosine deaminase deficiency, unspecified

        Inclusion term(s):

        • ADA deficiency NOS
      2. D81.31 Severe combined immunodeficiency due to adenosine deaminase deficiency

        Inclusion term(s):

        • ADA deficiency with SCID
        • Adenosine deaminase [ADA] deficiency with severe combined immunodeficiency
      3. D81.32 Adenosine deaminase 2 deficiency

        Inclusion term(s):

        • ADA2 deficiency
        • Adenosine deaminase deficiency type 2
      4. D81.39 Other adenosine deaminase deficiency

        Inclusion term(s):

        • Adenosine deaminase [ADA] deficiency type 1, NOS
        • Adenosine deaminase [ADA] deficiency type 1, without SCID
        • Adenosine deaminase [ADA] deficiency type 1, without severe combined immunodeficiency
        • Partial ADA deficiency (type 1)
        • Partial adenosine deaminase deficiency (type 1)
    5. D81.4 Nezelof's syndrome

    6. D81.5 Purine nucleoside phosphorylase [PNP] deficiency

    7. D81.6 Major histocompatibility complex class I deficiency

      Inclusion term(s):

      • Bare lymphocyte syndrome
    8. D81.7 Major histocompatibility complex class II deficiency

    9. D81.8 Other combined immunodeficiencies

      1. D81.81 Biotin-dependent carboxylase deficiency

        Inclusion term(s):

        • Multiple carboxylase deficiency
        • D81.810 Biotinidase deficiency
        • D81.818 Other biotin-dependent carboxylase deficiency

          Inclusion term(s):

          • Holocarboxylase synthetase deficiency
          • Other multiple carboxylase deficiency
        • D81.819 Biotin-dependent carboxylase deficiency, unspecified

          Inclusion term(s):

          • Multiple carboxylase deficiency, unspecified
      2. D81.89 Other combined immunodeficiencies

    10. D81.9 Combined immunodeficiency, unspecified

      Inclusion term(s):

      • Severe combined immunodeficiency disorder [SCID] NOS
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.