Certain disorders involving the immune mechanism (D80-D89)

Includes: defects in the complement system immunodeficiency disorders, except human immunodeficiency virus [HIV]

disease sarcoidosis

Excludes: autoimmune disease (systemic) NOS ( M35.9 )

functional disorders of polymorphonuclear neutrophils ( D71 ) human immunodeficiency virus [HIV] disease ( B20-B24 )

_ Immunodeficiency with predominantly antibody defects

D80.0 Hereditary hypogammaglobulinaemia

Autosomal recessive agammaglobulinaemia (Swiss type)

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

D80.1 Nonfamilial hypogammaglobulinaemia

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

Kappa light chain deficiency

D80.9 Immunodeficiency with predominantly antibody defects, unspecified

Combined immunodeficiencies

Excludes: 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'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

Biotin-dependent carboxylase deficiency D81.9 Combined immunodeficiency, unspecified

Severe combined immunodeficiency disorder [SCID] NOS

_ Immunodeficiency associated with other major defects

Excludes: 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

■ 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

_ 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

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 D84.9 Immunodeficiency, unspecified

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

Iridocyclitis in sarcoidosis+ ( H22.1* )

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


■ myositis+ ( M63.3* ) Uveoparotid fever [Heerfordt]

D86.9 Sarcoidosis, unspecified

Other disorders involving the immune mechanism, not elsewhere classified

Excludes: hyperglobulinaemia NOS ( R77.1 ) monoclonal gammopathy ( D47.2 ) transplant failure and rejection ( T86.

D89.0 Polyclonal hypergammaglobulinaemia

Benign hypergammaglobulinaemic purpura Polyclonal gammopathy NOS

D89.1 Cryoglobulinaemia


■ idiopathic

■ secondary Cryoglobulinaemic:

■ vasculitis


Hypergammaglobulinaemia, unspecified

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

D89.9 Disorder involving the immune mechanism, unspecified

Immune disease NOS

Processed on 05.04.2006 M. Source: World Health © Copyright WHO/DIMDI

S. Organization 1994/2006

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