Category: D81 - Combined immunodeficiencies

 Chapter Notes

 Section Notes

 D81

Combined immunodeficiencies

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

Code also, 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

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

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)

 D81.89

Other combined immunodeficiencies

 D81.9

Combined immunodeficiency, unspecified

Severe combined immunodeficiency disorder [SCID] NOS