D80 Immunodeficiency with predominantly antibody defects

D80.0 Hereditary hypogammaglobulinaemia

Incl.:

Autosomal recessive agammaglobulinaemia (Swiss type)

X-linked agammaglobulinaemia [Bruton] (with growth hormone deficiency)

D80.1 Nonfamilial hypogammaglobulinaemia

Incl.:

Agammaglobulinaemia with immunoglobulin-bearing B-lymphocytes

Common variable agammaglobulinaemia [CVAgamma]

Hypogammaglobulinaemia 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 hyperimmunoglobulinaemia

D80.7 Transient hypogammaglobulinaemia of infancy

D80.8 Other immunodeficiencies with predominantly antibody defects

Incl.:

Kappa light chain deficiency

D80.9 Immunodeficiency with predominantly antibody defects, unspecified

D81 Combined immunodeficiencies

Excl.:

autosomal recessive agammaglobulinaemia (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

D81.4 Nezelof syndrome

D81.5 Purine nucleoside phosphorylase [PNP] deficiency

D81.6 Major histocompatibility complex class I deficiency

Incl.:

Bare lymphocyte syndrome

D81.7 Major histocompatibility complex class II deficiency

D81.8 Other combined immunodeficiencies

Incl.:

Biotin-dependent carboxylase deficiency

D81.9 Combined immunodeficiency, unspecified

Incl.:

Severe combined immunodeficiency disorder [SCID] NOS

D82 Immunodeficiency associated with other major defects

Excl.:

ataxia telangiectasia [Louis-Bar] ( G11.3)

D82.0 Wiskott-Aldrich syndrome

Incl.:

Immunodeficiency with thrombocytopenia and eczema

D82.1 Di George syndrome

Incl.:

Pharyngeal pouch syndrome

Thymic:

• alymphoplasia
• aplasia or hypoplasia with immunodeficiency

D82.2 Immunodeficiency with short-limbed stature

D82.3 Immunodeficiency following hereditary defective response to Epstein-Barr virus

Incl.:

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

Incl.:

C1 esterase inhibitor [C1-INH] deficiency

D84.8 Other specified immunodeficiencies

D84.9 Immunodeficiency, unspecified

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 and combined sites

Incl.:

Iridocyclitis in sarcoidosis† ( H22.1*)

Multiple cranial nerve palsies in sarcoidosis† ( G53.2*)

Sarcoid:

• arthropathy† (M14.8*)
• myocarditis† (I41.8*)
• myositis† (M63.3*)

Uveoparotid fever [Heerfordt]

D86.9 Sarcoidosis, unspecified

D89 Other disorders involving the immune mechanism, not elsewhere classified

Excl.:

hyperglobulinaemia NOS ( R77.1)

monoclonal gammopathy of undertermined significance [MGUS] ( D47.2)

transplant failure and rejection ( T86.-)

D89.0 Polyclonal hypergammaglobulinaemia

Incl.:

Benign hypergammaglobulinaemic purpura

Polyclonal gammopathy NOS

D89.1 Cryoglobulinaemia

Incl.:

Cryoglobulinaemia:

• essential
• idiopathic
• mixed
• primary
• secondary

Cryoglobulinaemic:

• purpura
• vasculitis

D89.2 Hypergammaglobulinaemia, unspecified

D89.3 Immune reconstitution syndrome

Incl.:

Immune reconstitution inflammatory syndrome [IRIS]

Use additional external cause code (Chapter XX), if desired, to identify drug.

D89.8 Other specified disorders involving the immune mechanism, not elsewhere classified

D89.9 Disorder involving the immune mechanism, unspecified

Incl.:

Immune disease NOS