ICD-10-CM Book 2024 Edition

Chapters  /  Chapter 3  /  D80-D89

Certain disorders involving the immune mechanism (D80-D89)

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)
D80 Immunodeficiency with predominantly antibody defects
D80.0 Hereditary hypogammaglobulinemia
Autosomal recessive agammaglobulinemia (Swiss type)
X-linked agammaglobulinemia [Bruton] (with growth hormone deficiency)
D80.1 Nonfamilial hypogammaglobulinemia
Agammaglobulinemia with immunoglobulin-bearing B-lymphocytes
Common variable agammaglobulinemia [CVAgamma]
Hypogammaglobulinemia NOS
D80.2 Selective deficiency of immunoglobulin A [IgA]
D80.3 Selective deficiency of immunoglobulin G [IgG] subclasses
D80.4 Selective deficiency of immunoglobulin M [IgM]
D80.5 Immunodeficiency with increased immunoglobulin M [IgM]
D80.6 Antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinemia
D80.7 Transient hypogammaglobulinemia of infancy
D80.8 Other immunodeficiencies with predominantly antibody defects
Kappa light chain deficiency
D80.9 Immunodeficiency with predominantly antibody defects, unspecified
D81 Combined immunodeficiencies
Excludes1 autosomal recessive agammaglobulinemia (Swiss type) (D80.0)
D81.0 Severe combined immunodeficiency [SCID] with reticular dysgenesis
D81.1 Severe combined immunodeficiency [SCID] with low T- and B-cell numbers
D81.2 Severe combined immunodeficiency [SCID] with low or normal B-cell numbers
D81.3 Adenosine deaminase [ADA] deficiency
D81.30 Adenosine deaminase deficiency, unspecified
ADA deficiency NOS
D81.31 Severe combined immunodeficiency due to adenosine deaminase deficiency
ADA deficiency with SCID
Adenosine deaminase [ADA] deficiency with severe combined immunodeficiency
D81.32 Adenosine deaminase 2 deficiency
ADA2 deficiency
Adenosine deaminase deficiency type 2
CodeAlso , if applicable, any associated manifestations, such as:
polyarteritis nodosa (M30.0)
stroke (I63.-)
D81.39 Other adenosine deaminase deficiency
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)
D81.4 Nezelof's syndrome
D81.5 Purine nucleoside phosphorylase [PNP] deficiency
D81.6 Major histocompatibility complex class I deficiency
Bare lymphocyte syndrome
D81.7 Major histocompatibility complex class II deficiency
D81.8 Other combined immunodeficiencies
D81.81 Biotin-dependent carboxylase deficiency
Multiple carboxylase deficiency
Excludes1 biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)
D81.810 Biotinidase deficiency
D81.818 Other biotin-dependent carboxylase deficiency
Holocarboxylase synthetase deficiency
Other multiple carboxylase deficiency
D81.819 Biotin-dependent carboxylase deficiency, unspecified
Multiple carboxylase deficiency, unspecified
D81.82 Activated Phosphoinositide 3-kinase Delta Syndrome [APDS]
p110d-activating mutation causing senescent T cells, lymphadenopathy, and immunodeficiency [PASLI] disease
CodeAlso , if applicable, any associated manifestations, such as:
bronchiectasis (J47.-)
herpes virus infections (B00.-)
other acute respiratory tract infections (J00-J06; J20-J22)
other infections (A00-B99)
pneumonia (J12-J18)
D81.89 Other combined immunodeficiencies
D81.9 Combined immunodeficiency, unspecified
Severe combined immunodeficiency disorder [SCID] NOS
D82 Immunodeficiency associated with other major defects
Excludes1 ataxia telangiectasia [Louis-Bar] (G11.3)
D82.0 Wiskott-Aldrich syndrome
Immunodeficiency with thrombocytopenia and eczema
D82.1 Di George's syndrome
Pharyngeal pouch syndrome
Thymic alymphoplasia
Thymic aplasia or hypoplasia with immunodeficiency
D82.2 Immunodeficiency with short-limbed stature
D82.3 Immunodeficiency following hereditary defective response to Epstein-Barr virus
X-linked lymphoproliferative disease
D82.4 Hyperimmunoglobulin E [IgE] syndrome
D82.8 Immunodeficiency associated with other specified major defects
D82.9 Immunodeficiency associated with major defect, unspecified
D83 Common variable immunodeficiency
D83.0 Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function
D83.1 Common variable immunodeficiency with predominant immunoregulatory T-cell disorders
D83.2 Common variable immunodeficiency with autoantibodies to B- or T-cells
D83.8 Other common variable immunodeficiencies
D83.9 Common variable immunodeficiency, unspecified
D84 Other immunodeficiencies
D84.0 Lymphocyte function antigen-1 [LFA-1] defect
D84.1 Defects in the complement system
C1 esterase inhibitor [C1-INH] deficiency
D84.8 Other specified immunodeficiencies
D84.81 Immunodeficiency due to conditions classified elsewhere
CodeFirst underlying condition, such as:
chromosomal abnormalities (Q90-Q99)
diabetes mellitus (E08-E13)
malignant neoplasms (C00-C96)
Excludes1 certain disorders involving the immune mechanism (D80-D83, D84.0, D84.1, D84.9)
human immunodeficiency virus [HIV] disease (B20)
D84.82 Immunodeficiency due to drugs and external causes
D84.821 Immunodeficiency due to drugs
Immunodeficiency due to (current or past) medication
UseAdditionalCode code for adverse effect if applicable, to identify adverse effect of drug (T36-T50 with fifth or six character 5)
UseAdditionalCode code, if applicable, for associated long term (current) drug therapy drug or medication such as:
long term (current) drug therapy systemic steroids (Z79.52)
other long term (current) drug therapy (Z79.899)
D84.822 Immunodeficiency due to external causes
CodeAlso , if applicable, radiological procedure and radiotherapy (Y84.2)
UseAdditionalCode code for external cause such as:
exposure to ionizing radiation (W88)
D84.89 Other immunodeficiencies
D84.9 Immunodeficiency, unspecified
Immunocompromised NOS
Immunodeficient NOS
Immunosuppressed NOS
D86 Sarcoidosis
D86.0 Sarcoidosis of lung
D86.1 Sarcoidosis of lymph nodes
D86.2 Sarcoidosis of lung with sarcoidosis of lymph nodes
D86.3 Sarcoidosis of skin
D86.8 Sarcoidosis of other sites
D86.81 Sarcoid meningitis
D86.82 Multiple cranial nerve palsies in sarcoidosis
D86.83 Sarcoid iridocyclitis
D86.84 Sarcoid pyelonephritis
Tubulo-interstitial nephropathy in sarcoidosis
D86.85 Sarcoid myocarditis
D86.86 Sarcoid arthropathy
Polyarthritis in sarcoidosis
D86.87 Sarcoid myositis
D86.89 Sarcoidosis of other sites
Hepatic granuloma
Uveoparotid fever [Heerfordt]
D86.9 Sarcoidosis, unspecified
D89 Other disorders involving the immune mechanism, not elsewhere classified
Excludes1 hyperglobulinemia NOS (R77.1)
monoclonal gammopathy (of undetermined significance) (D47.2)
Excludes2 transplant failure and rejection (T86.-)
D89.0 Polyclonal hypergammaglobulinemia
Benign hypergammaglobulinemic purpura
Polyclonal gammopathy NOS
D89.1 Cryoglobulinemia
Cryoglobulinemic purpura
Cryoglobulinemic vasculitis
Essential cryoglobulinemia
Idiopathic cryoglobulinemia
Mixed cryoglobulinemia
Primary cryoglobulinemia
Secondary cryoglobulinemia
D89.2 Hypergammaglobulinemia, unspecified
D89.3 Immune reconstitution syndrome
Immune reconstitution inflammatory syndrome [IRIS]
UseAdditionalCode code for adverse effect, if applicable, to identify drug (T36-T50 with fifth or sixth character 5)
D89.4 Mast cell activation syndrome and related disorders
Excludes1 aggressive systemic mastocytosis (C96.21)
congenital cutaneous mastocytosis (Q82.2)
(non-congenital) cutaneous mastocytosis (D47.01)
(indolent) systemic mastocytosis (D47.02)
malignant mast cell neoplasm (C96.2-)
malignant mastocytoma (C96.29)
mast cell leukemia (C94.3-)
mast cell sarcoma (C96.22)
mastocytoma NOS (D47.09)
other mast cell neoplasms of uncertain behavior (D47.09)
systemic mastocytosis associated with a clonal hematologic non-mast cell lineage disease (SM-AHNMD) (D47.02)
D89.40 Mast cell activation, unspecified
Mast cell activation disorder, unspecified
Mast cell activation syndrome, NOS
D89.41 Monoclonal mast cell activation syndrome
D89.42 Idiopathic mast cell activation syndrome
D89.43 Secondary mast cell activation
Secondary mast cell activation syndrome
CodeAlso underlying etiology, if known
D89.44 Hereditary alpha tryptasemia
UseAdditionalCode code, if applicable, for:
allergy status, other than to drugs and biological substances (Z91.0-)
personal history of anaphylaxis (Z87.892)
D89.49 Other mast cell activation disorder
Other mast cell activation syndrome
D89.8 Other specified disorders involving the immune mechanism, not elsewhere classified
D89.81 Graft-versus-host disease
CodeFirst underlying cause, such as:
complications of transplanted organs and tissue (T86.-)
complications of blood transfusion (T80.89)
UseAdditionalCode code to identify associated manifestations, such as:
desquamative dermatitis (L30.8)
diarrhea (R19.7)
elevated bilirubin (R17)
hair loss (L65.9)
D89.810 Acute graft-versus-host disease
nonPrimary Yes
D89.811 Chronic graft-versus-host disease
nonPrimary Yes
D89.812 Acute on chronic graft-versus-host disease
nonPrimary Yes
D89.813 Graft-versus-host disease, unspecified
nonPrimary Yes
D89.82 Autoimmune lymphoproliferative syndrome [ALPS]
D89.83 Cytokine release syndrome
CodeFirst underlying cause, such as:
complications following infusion, transfusion and therapeutic injection (T80.89-)
complications of transplanted organs and tissue (T86.-)
UseAdditionalCode code to identify associated manifestations
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
D89.84 IgG4-related disease
Immunoglobulin G4-related disease
D89.89 Other specified disorders involving the immune mechanism, not elsewhere classified
Excludes1 human immunodeficiency virus disease (B20)
D89.9 Disorder involving the immune mechanism, unspecified
Immune disease NOS