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


    • hyperglobulinemia NOS (R77.1)
    • monoclonal gammopathy (of undetermined significance) (D47.2)


    • transplant failure and rejection (T86.-)
    1. D89.0 Polyclonal hypergammaglobulinemia

      Inclusion term(s):

      • Benign hypergammaglobulinemic purpura
      • Polyclonal gammopathy NOS
    2. D89.1 Cryoglobulinemia

      Inclusion term(s):

      • Cryoglobulinemic purpura
      • Cryoglobulinemic vasculitis
      • Essential cryoglobulinemia
      • Idiopathic cryoglobulinemia
      • Mixed cryoglobulinemia
      • Primary cryoglobulinemia
      • Secondary cryoglobulinemia
    3. D89.2 Hypergammaglobulinemia, unspecified

    4. D89.3 Immune reconstitution syndrome

      Inclusion term(s):

      • Immune reconstitution inflammatory syndrome [IRIS]
    5. D89.4 Mast cell activation syndrome and related disorders

      1. D89.40 Mast cell activation, unspecified

        Inclusion term(s):

        • Mast cell activation disorder, unspecified
        • Mast cell activation syndrome, NOS
      2. D89.41 Monoclonal mast cell activation syndrome

      3. D89.42 Idiopathic mast cell activation syndrome

      4. D89.43 Secondary mast cell activation

        Inclusion term(s):

        • Secondary mast cell activation syndrome
      5. D89.44 Hereditary alpha tryptasemia

      6. D89.49 Other mast cell activation disorder

        Inclusion term(s):

        • Other mast cell activation syndrome
    6. D89.8 Other specified disorders involving the immune mechanism, not elsewhere classified

      1. D89.81 Graft-versus-host disease

        • D89.810 Acute graft-versus-host disease
        • D89.811 Chronic graft-versus-host disease
        • D89.812 Acute on chronic graft-versus-host disease
        • D89.813 Graft-versus-host disease, unspecified
      2. D89.82 Autoimmune lymphoproliferative syndrome [ALPS]

      3. D89.83 Cytokine release syndrome

        • D89.831 Cytokine release syndrome, grade 1
        • D89.832 Cytokine release syndrome, grade 2
        • D89.833 Cytokine release syndrome, grade 3
        • D89.834 Cytokine release syndrome, grade 4
        • D89.835 Cytokine release syndrome, grade 5
        • D89.839 Cytokine release syndrome, grade unspecified
      4. D89.84 IgG4-related disease

        Inclusion term(s):

        • Immunoglobulin G4-related disease
      5. D89.89 Other specified disorders involving the immune mechanism, not elsewhere classified

    7. D89.9 Disorder involving the immune mechanism, unspecified

      Inclusion term(s):

      • Immune disease 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.