Chapter III

Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism

Certain disorders involving the immune mechanism

Includes: defects in the complement system
immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
Excludes: autoimmune disease (systemic) NOS ( M35.9 )
functional disorders of polymorphonuclear neutrophils ( D71 )
human immunodeficiency virus [HIV] disease ( B20-B24 )

D80 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

D81 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

D82 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
· 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

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
D84.9 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
Iridocyclitis in sarcoidosis+ ( H22.1* )
Multiple cranial nerve palsies in sarcoidosis+ ( G53.2* )
· 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
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
· essential
· idiopathic
· mixed
· primary
· secondary
· purpura
· vasculitis
D89.2 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