Certain disorders involving the immune mechanism (D80-D89)
D80Immunodeficiency with predominantly antibody defects
D80.0Hereditary hypogammaglobulinemia
› Autosomal recessive agammaglobulinemia (Swiss type)
› X-linked agammaglobulinemia [Bruton] (with growth hormone deficiency)
D80.1Nonfamilial hypogammaglobulinemia
› Agammaglobulinemia with immunoglobulin-bearing B-lymphocytes
› Common variable agammaglobulinemia [CVAgamma]
› Hypogammaglobulinemia NOS
D80.2Selective deficiency of immunoglobulin A [IgA]
D80.3Selective deficiency of immunoglobulin G [IgG] subclasses
D80.4Selective deficiency of immunoglobulin M [IgM]
D80.5Immunodeficiency with increased immunoglobulin M [IgM]
D80.6Antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinemia
D80.7Transient hypogammaglobulinemia of infancy
D80.8Other immunodeficiencies with predominantly antibody defects
› Kappa light chain deficiency
D80.9Immunodeficiency with predominantly antibody defects, unspecified
D81Combined immunodeficiencies
Excludes 1: autosomal recessive agammaglobulinemia (Swiss type) (D80.0)
D81.0Severe combined immunodeficiency [SCID] with reticular dysgenesis
D81.1Severe combined immunodeficiency [SCID] with low T- and B-cell numbers
D81.2Severe combined immunodeficiency [SCID] with low or normal B-cell numbers
D81.3Adenosine deaminase [ADA] deficiency
D81.30Adenosine deaminase deficiency, unspecified
› ADA deficiency NOS
D81.31Severe combined immunodeficiency due to adenosine deaminase deficiency
› ADA deficiency with SCID
› Adenosine deaminase [ADA] deficiency with severe combined immunodeficiency
D81.32Adenosine deaminase 2 deficiency
› ADA2 deficiency
› Adenosine deaminase deficiency type 2
Code Also: , if applicable, any associated manifestations, such as:
› polyarteritis nodosa (M30.0)
› stroke (I63.-)
D81.39Other 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)
D81.4Nezelof's syndrome
D81.5Purine nucleoside phosphorylase [PNP] deficiency
D81.6Major histocompatibility complex class I deficiency
› Bare lymphocyte syndrome
D81.7Major histocompatibility complex class II deficiency
D81.8Other combined immunodeficiencies
D81.81Biotin-dependent carboxylase deficiency
› Multiple carboxylase deficiency
Excludes 1: biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)
D81.810Biotinidase deficiency
D81.818Other biotin-dependent carboxylase deficiency
› Holocarboxylase synthetase deficiency
› Other multiple carboxylase deficiency
D81.819Biotin-dependent carboxylase deficiency, unspecified
› Multiple carboxylase deficiency, unspecified
D81.82Activated Phosphoinositide 3-kinase Delta Syndrome [APDS]
› p110d-activating mutation causing senescent T cells, lymphadenopathy, and immunodeficiency [PASLI] disease
Code Also: , 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)
D81.89Other combined immunodeficiencies
D81.9Combined immunodeficiency, unspecified
› Severe combined immunodeficiency disorder [SCID] NOS
D82Immunodeficiency associated with other major defects
Excludes 1: ataxia telangiectasia [Louis-Bar] (G11.3)
D82.0Wiskott-Aldrich syndrome
› Immunodeficiency with thrombocytopenia and eczema
D82.1Di George's syndrome
› Pharyngeal pouch syndrome
› Thymic alymphoplasia
› Thymic aplasia or hypoplasia with immunodeficiency
D82.2Immunodeficiency with short-limbed stature
D82.3Immunodeficiency following hereditary defective response to Epstein-Barr virus
› X-linked lymphoproliferative disease
D82.4Hyperimmunoglobulin E [IgE] syndrome
D82.8Immunodeficiency associated with other specified major defects
D82.9Immunodeficiency associated with major defect, unspecified
D83Common variable immunodeficiency
D83.0Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function
D83.1Common variable immunodeficiency with predominant immunoregulatory T-cell disorders
D83.2Common variable immunodeficiency with autoantibodies to B- or T-cells
D83.8Other common variable immunodeficiencies
D83.9Common variable immunodeficiency, unspecified
D84Other immunodeficiencies
D84.0Lymphocyte function antigen-1 [LFA-1] defect
D84.1Defects in the complement system
› C1 esterase inhibitor [C1-INH] deficiency
D84.8Other specified immunodeficiencies
D84.81Immunodeficiency due to conditions classified elsewhere
Code First: underlying condition, such as:
› chromosomal abnormalities (Q90-Q99)
› diabetes mellitus (E08-E13)
› malignant neoplasms (C00-C96)
Excludes 1: certain disorders involving the immune mechanism (D80-D83, D84.0, D84.1, D84.9)
› human immunodeficiency virus [HIV] disease (B20)
D84.82Immunodeficiency due to drugs and external causes
D84.821Immunodeficiency due to drugs
› Immunodeficiency due to (current or past) medication
Use Additional Code: code for adverse effect if applicable, to identify adverse effect of drug (T36-T50 with fifth or six character 5)
Use Additional Code: 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)
D84.822Immunodeficiency due to external causes
Code Also: , if applicable, radiological procedure and radiotherapy (Y84.2)
Use Additional Code: code for external cause such as:
› exposure to ionizing radiation (W88)
D84.89Other immunodeficiencies
D84.9Immunodeficiency, unspecified
› Immunocompromised NOS
› Immunodeficient NOS
› Immunosuppressed NOS
D86Sarcoidosis
D86.0Sarcoidosis of lung
D86.1Sarcoidosis of lymph nodes
D86.2Sarcoidosis of lung with sarcoidosis of lymph nodes
D86.3Sarcoidosis of skin
D86.8Sarcoidosis of other sites
D86.81Sarcoid meningitis
D86.82Multiple cranial nerve palsies in sarcoidosis
D86.83Sarcoid iridocyclitis
D86.84Sarcoid pyelonephritis
› Tubulo-interstitial nephropathy in sarcoidosis
D86.85Sarcoid myocarditis
D86.86Sarcoid arthropathy
› Polyarthritis in sarcoidosis
D86.87Sarcoid myositis
D86.89Sarcoidosis of other sites
› Hepatic granuloma
› Uveoparotid fever [Heerfordt]
D86.9Sarcoidosis, unspecified
D89Other disorders involving the immune mechanism, not elsewhere classified
Excludes 1: hyperglobulinemia NOS (R77.1)
› monoclonal gammopathy (of undetermined significance) (D47.2)
Excludes 2: transplant failure and rejection (T86.-)
D89.0Polyclonal hypergammaglobulinemia
› Benign hypergammaglobulinemic purpura
› Polyclonal gammopathy NOS
D89.1Cryoglobulinemia
› Cryoglobulinemic purpura
› Cryoglobulinemic vasculitis
› Essential cryoglobulinemia
› Idiopathic cryoglobulinemia
› Mixed cryoglobulinemia
› Primary cryoglobulinemia
› Secondary cryoglobulinemia
D89.2Hypergammaglobulinemia, unspecified
D89.3Immune reconstitution syndrome
› Immune reconstitution inflammatory syndrome [IRIS]
Use Additional Code: code for adverse effect, if applicable, to identify drug (T36-T50) with fifth or sixth character 5)
D89.4Mast cell activation syndrome and related disorders
Excludes 1: 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)
D89.40Mast cell activation, unspecified
› Mast cell activation disorder, unspecified
› Mast cell activation syndrome, NOS
D89.41Monoclonal mast cell activation syndrome
D89.42Idiopathic mast cell activation syndrome
D89.43Secondary mast cell activation
› Secondary mast cell activation syndrome
Code Also: underlying etiology, if known
D89.44Hereditary alpha tryptasemia
Use Additional Code: code, if applicable, for:
› allergy status, other than to drugs and biological substances (Z91.0-)
› personal history of anaphylaxis (Z87.892)
D89.49Other mast cell activation disorder
› Other mast cell activation syndrome
D89.8Other specified disorders involving the immune mechanism, not elsewhere classified
D89.81Graft-versus-host disease
Code First: underlying cause, such as:
› complications of transplanted organs and tissue (T86.-)
› complications of blood transfusion (T80.89)
Use Additional Code: code to identify associated manifestations, such as:
› desquamative dermatitis (L30.8)
› diarrhea (R19.7)
› elevated bilirubin (R17)
› hair loss (L65.9)
D89.810Acute graft-versus-host disease
D89.811Chronic graft-versus-host disease
D89.812Acute on chronic graft-versus-host disease
D89.813Graft-versus-host disease, unspecified
D89.82Autoimmune lymphoproliferative syndrome [ALPS]
D89.83Cytokine release syndrome
Code First: underlying cause, such as:
› complications following infusion, transfusion and therapeutic injection (T80.89-)
› complications of transplanted organs and tissue (T86.-)
Use Additional Code: code to identify associated manifestations
D89.831Cytokine release syndrome, grade 1
D89.832Cytokine release syndrome, grade 2
D89.833Cytokine release syndrome, grade 3
D89.834Cytokine release syndrome, grade 4
D89.835Cytokine release syndrome, grade 5
D89.839Cytokine release syndrome, grade unspecified
D89.84IgG4-related disease
› Immunoglobulin G4-related disease
D89.89Other specified disorders involving the immune mechanism, not elsewhere classified
Excludes 1: human immunodeficiency virus disease (B20)
D89.9Disorder involving the immune mechanism, unspecified
› Immune disease NOS