ICD-10-CM Book 2026 Edition

Chapters  /  Chapter 3  /  D80-D89  /  D81
D81 Combined immunodeficiencies
Excludes1 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
CodeAlso , 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
Excludes1 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
CodeAlso , 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