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A comprehensive list of ICD-10 and ICD-9 codes for various types of immunodeficiencies and related disorders, including functional disorders of polymorphonuclear neutrophils, immunodeficiency with predominantly antibody defects, combined immunodeficiencies, immunodeficiency associated with other major defects, common variable immunodeficiency, and other specified immunodeficiencies. The document also includes information on diagnostic related groups and applicable conditions.
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Applicable To: Cell membrane receptor complex [CR3] defect Chronic (childhood) granulomatous disease Congenital dysphagocytosis Progressive septic granulomatosis D71 is grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 808, 809, 810
Hereditary hypogammaglobulinemia
Applicable To: Autosomal recessive agammaglobulinemia (Swiss type) X-linked agammaglobulinemia [Bruton] (with growth hormone deficiency) D80. Nonfamilial hypogammaglobulinemia
Applicable To: Agammaglobulinemia with immunoglobulin-bearing B-lymphocytes Common variable agammaglobulinemia [CVAgamma] Hypogammaglobulinemia NOS D80. Selective deficiency of immunoglobulin A [IgA]
Selective deficiency of immunoglobulin G [IgG] subclasses
Selective deficiency of immunoglobulin M [IgM]
Immunodeficiency with increased immunoglobulin M [IgM]
D80 to D80.5 are grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 814, 815, 816 D80. Antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinemia
D80.6 is grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 808, 809 810 D80. Transient hypogammaglobulinemia of infancy
D80.7 is grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 814, 815, 816
Other immunodeficiencies with predominantly antibody defects
Applicable To: Kappa light chain deficiency D80. Immunodeficiency with predominantly antibody defects, unspecified
D80.8 and D80.9 are grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 808, 809 810
Severe combined immunodeficiency [SCID] with reticular dysgenesis
Severe combined immunodeficiency [SCID] with low T- and B-cell numbers
Severe combined immunodeficiency [SCID] with low or normal B-cell numbers
D81.0 to D81.2 are grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 808, 809 810 D81. Adenosine deaminase [ADA] deficiency
D81.3 is grouped within Diagnostic Related Group (MS-DRG v34.0): 642 D81. Nezelof's syndrome
D81.4 is grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 808, 809 810 D81. Purine nucleoside phosphorylase [PNP] deficiency
D81.5 is grouped within Diagnostic Related Group (MS-DRG v34.0): 642 D81. Major histocompatibility complex class I deficiency
Applicable To: Bare lymphocyte syndrome D81. Major histocompatibility complex class II deficiency
D81.6 and D81.7 are grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 808, 809 810 D81.
Biotin-dependent carboxylase deficiency (not billable, use one of the below codes) D81. Biotinidase deficiency
D81.810 is grouped within Diagnostic Related Group (MS-DRG v34.0): 642 D81. Other biotin-dependent carboxylase deficiency
Applicable To: Holocarboxylase synthetase deficiency Holocarboxylase synthetase deficiency D81. Other biotin-dependent carboxylase deficiency, unspecified
Applicable To: Multiple carboxylase deficiency, unspecified
Lymphocyte function antigen-1 [LFA-1] defect
D84.0 is grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 814, 815, 816 D84. Defects in the complement system
Applicable To: C1 estrase inhibitor [C1-INH] deficiency (not inherited deficiency in complement components) D84.1 is grouped within Diagnostic Related Group (MS-DRG v34.0): 642 D84. Other specified immunodeficiencies 279.10 or
Immunodeficiency, unspecified
D84.8 and D84.9 are grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 814, 815, 816
Polyclonal hypergammaglobulinemia
Applicable To: Benign hypergammaglobulinemic purpura Polyclonal gammopathy NOS D89.0 is grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 814, 815, 816 D89. Cryoglobulinemia
Applicable To: Cryoglobulinemic purpura Cryoglobulinemic vasculitis Essential cryoglobulinemia Idiopathic cryoglobulinemia Mixed cryoglobulinemia Primary cryoglobulinemia Secondary cryoglobulinemia D89.1 is grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 820 to 825, 840 to 842 D89. Hypergammaglobulinemia, unspecified 273.1 or
Immune reconstitution syndrome
Applicable To: Immune reconsitution inflammatory syndrome {IRIS} Use additional code for adverse effect, if applicable to identify drug (T36-T50) with fifth or sixth character 5) D89.2 and D89.3 are grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 814, 815, 816
Mast cell activation syndrome and related disorders (not billable, use one of the below codes) D89. Mast cell activation, unspecified
Applicable To: Mast cell activation disorder, unspecified Mast cell activation syndrome, NOS D89. Monoclonal mast cell activation syndrome
Idiopathic mast cell activation syndrome
Secondary mast cell activation
Applicable To: Secondary mast cell activation syndrome Code also underlying etiology, if known D89. Other mast cell activation disorder Applicable To:
Other mast cell activation syndrome D89.40 to D89.43 and D89.49 are grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 814, 815, 816 D89.
Graft-versus-host disease (not billable, use one of the below codes) D89. Acute graft-versus-host disease
Chronic graft-versus-host disease
Acute on chronic graft-versus-host disease
Graft-versus-host disease, unspecified
D89.10 to D89.13 are are grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 808, 809, 810 D89. Autoimmune lymphoproliferative syndrome [ALPS]
D89.82 is grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 545, 546, 547 D89. Other specified disorders involving the immune mechanism, not elsewhere classified
Type 1 Excludes: Human immunodeficiency virus disease (B20) D89.89 is grouped within Diagnostic Related Group(s) (MS-DRG v34.0): D89. Disorder involving the immune mechanism, unspecified
Applicable To: Immune disease NOS D89.9 is grouped within Diagnostic Related Group(s) (MS-DRG v34.0): 814, 815, 816 3/