ICD-10-CM Section · D80-D89
Certain disorders involving the immune mechanism
100 codes in this section
100
Total Codes
83
Billable
17
Header (Non-Billable)
7
Top-Level Codes
About Section D80-D89
Section D80-D89 (D80-D89) covers certain disorders involving the immune mechanism. It sits within the Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism chapter (D50-D89). This section contains 100 ICD-10-CM codes, 83 billable diagnoses you can submit on a claim, and 17 non-billable header codes used to organize the hierarchy.
Codes are organized below by 3-character root. Click the root to see all subcodes underneath it, or click any individual code for full coverage data, payer policies, and linked CPT procedures.
Codes in D80-D89
Grouped by 3-character root code. Click any code for full detail.
D80
Immunodeficiency with predominantly antibody defects
10 subcodes
| D80.0 | Hereditary hypogammaglobulinemia | Billable |
| D80.1 | Nonfamilial hypogammaglobulinemia | Billable |
| D80.2 | Selective deficiency of immunoglobulin A [IgA] | Billable |
| D80.3 | Selective deficiency of immunoglobulin G [IgG] subclasses | Billable |
| D80.4 | Selective deficiency of immunoglobulin M [IgM] | Billable |
| D80.5 | Immunodeficiency with increased immunoglobulin M [IgM] | Billable |
| D80.6 | Antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinemia | Billable |
| D80.7 | Transient hypogammaglobulinemia of infancy | Billable |
| D80.8 | Other immunodeficiencies with predominantly antibody defects | Billable |
| D80.9 | Immunodeficiency with predominantly antibody defects, unspecified | Billable |
D81
Combined immunodeficiencies
20 subcodes
| D81.0 | Severe combined immunodeficiency [SCID] with reticular dysgenesis | Billable |
| D81.1 | Severe combined immunodeficiency [SCID] with low T- and B-cell numbers | Billable |
| D81.2 | Severe combined immunodeficiency [SCID] with low or normal B-cell numbers | Billable |
| D81.3 | Adenosine deaminase [ADA] deficiency | Header |
| D81.30 | Adenosine deaminase deficiency, unspecified | Billable |
| D81.31 | Severe combined immunodeficiency due to adenosine deaminase deficiency | Billable |
| D81.32 | Adenosine deaminase 2 deficiency | Billable |
| D81.39 | Other adenosine deaminase deficiency | Billable |
| D81.4 | Nezelof's syndrome | Billable |
| D81.5 | Purine nucleoside phosphorylase [PNP] deficiency | Billable |
| D81.6 | Major histocompatibility complex class I deficiency | Billable |
| D81.7 | Major histocompatibility complex class II deficiency | Billable |
| D81.8 | Other combined immunodeficiencies | Header |
| D81.81 | Biotin-dependent carboxylase deficiency | Header |
| D81.810 | Biotinidase deficiency | Billable |
| D81.818 | Other biotin-dependent carboxylase deficiency | Billable |
| D81.819 | Biotin-dependent carboxylase deficiency, unspecified | Billable |
| D81.82 | Activated Phosphoinositide 3-kinase Delta Syndrome [APDS] | Billable |
| D81.89 | Other combined immunodeficiencies | Billable |
| D81.9 | Combined immunodeficiency, unspecified | Billable |
D82
Immunodeficiency associated with other major defects
7 subcodes
| D82.0 | Wiskott-Aldrich syndrome | Billable |
| D82.1 | Di George's syndrome | Billable |
| D82.2 | Immunodeficiency with short-limbed stature | Billable |
| D82.3 | Immunodeficiency following hereditary defective response to Epstein-Barr virus | Billable |
| D82.4 | Hyperimmunoglobulin E [IgE] syndrome | Billable |
| D82.8 | Immunodeficiency associated with other specified major defects | Billable |
| D82.9 | Immunodeficiency associated with major defect, unspecified | Billable |
D83
Common variable immunodeficiency
5 subcodes
| D83.0 | Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function | Billable |
| D83.1 | Common variable immunodeficiency with predominant immunoregulatory T-cell disorders | Billable |
| D83.2 | Common variable immunodeficiency with autoantibodies to B- or T-cells | Billable |
| D83.8 | Other common variable immunodeficiencies | Billable |
| D83.9 | Common variable immunodeficiency, unspecified | Billable |
D84
Other immunodeficiencies
9 subcodes
| D84.0 | Lymphocyte function antigen-1 [LFA-1] defect | Billable |
| D84.1 | Defects in the complement system | Billable |
| D84.8 | Other specified immunodeficiencies | Header |
| D84.81 | Immunodeficiency due to conditions classified elsewhere | Billable |
| D84.82 | Immunodeficiency due to drugs and external causes | Header |
| D84.821 | Immunodeficiency due to drugs | Billable |
| D84.822 | Immunodeficiency due to external causes | Billable |
| D84.89 | Other immunodeficiencies | Billable |
| D84.9 | Immunodeficiency, unspecified | Billable |
D86
Sarcoidosis
14 subcodes
| D86.0 | Sarcoidosis of lung | Billable |
| D86.1 | Sarcoidosis of lymph nodes | Billable |
| D86.2 | Sarcoidosis of lung with sarcoidosis of lymph nodes | Billable |
| D86.3 | Sarcoidosis of skin | Billable |
| D86.8 | Sarcoidosis of other sites | Header |
| D86.81 | Sarcoid meningitis | Billable |
| D86.82 | Multiple cranial nerve palsies in sarcoidosis | Billable |
| D86.83 | Sarcoid iridocyclitis | Billable |
| D86.84 | Sarcoid pyelonephritis | Billable |
| D86.85 | Sarcoid myocarditis | Billable |
| D86.86 | Sarcoid arthropathy | Billable |
| D86.87 | Sarcoid myositis | Billable |
| D86.89 | Sarcoidosis of other sites | Billable |
| D86.9 | Sarcoidosis, unspecified | Billable |
D89
Other disorders involving the immune mechanism, not elsewhere classified
28 subcodes
| D89.0 | Polyclonal hypergammaglobulinemia | Billable |
| D89.1 | Cryoglobulinemia | Billable |
| D89.2 | Hypergammaglobulinemia, unspecified | Billable |
| D89.3 | Immune reconstitution syndrome | Billable |
| D89.4 | Mast cell activation syndrome and related disorders | Header |
| D89.40 | Mast cell activation, unspecified | Billable |
| D89.41 | Monoclonal mast cell activation syndrome | Billable |
| D89.42 | Idiopathic mast cell activation syndrome | Billable |
| D89.43 | Secondary mast cell activation | Billable |
| D89.44 | Hereditary alpha tryptasemia | Billable |
| D89.49 | Other mast cell activation disorder | Billable |
| D89.8 | Other specified disorders involving the immune mechanism, not elsewhere classified | Header |
| D89.81 | Graft-versus-host disease | Header |
| D89.810 | Acute graft-versus-host disease | Billable |
| D89.811 | Chronic graft-versus-host disease | Billable |
| D89.812 | Acute on chronic graft-versus-host disease | Billable |
| D89.813 | Graft-versus-host disease, unspecified | Billable |
| D89.82 | Autoimmune lymphoproliferative syndrome [ALPS] | Billable |
| D89.83 | Cytokine release syndrome | Header |
| D89.831 | Cytokine release syndrome, grade 1 | Billable |
| D89.832 | Cytokine release syndrome, grade 2 | Billable |
| D89.833 | Cytokine release syndrome, grade 3 | Billable |
| D89.834 | Cytokine release syndrome, grade 4 | Billable |
| D89.835 | Cytokine release syndrome, grade 5 | Billable |
| D89.839 | Cytokine release syndrome, grade unspecified | Billable |
| D89.84 | IgG4-related disease | Billable |
| D89.89 | Other specified disorders involving the immune mechanism, not elsewhere classified | Billable |
| D89.9 | Disorder involving the immune mechanism, unspecified | Billable |
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Verified against the CMS 2026 code set on May 31, 2026.
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