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D81 Combined immunodeficiencies

ICD-10-CM category D81 sits in Chapter 3 (Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism, D50-D89). It contains 17 billable codes for FY2027, effective October 1, 2026 to September 30, 2027. Always code to the most specific code available: a code with more characters that describes the diagnosis more precisely takes priority over a shorter one.

Subcategories

D81.3 Adenosine deaminase [ADA] deficiencyD81.8 Other combined immunodeficienciesD81.81 Biotin-dependent carboxylase deficiency

Billable codes in D81

CodeDescription
D81.0Severe combined immunodeficiency [SCID] with reticular dysgenesis
D81.1Severe combined immunodeficiency [SCID] with low T- and B-cell numbers
D81.2Severe combined immunodeficiency [SCID] with low or normal B-cell numbers
D81.30Adenosine deaminase deficiency, unspecified
D81.31Severe combined immunodeficiency due to adenosine deaminase deficiency
D81.32Adenosine deaminase 2 deficiency
D81.39Other adenosine deaminase deficiency
D81.4Nezelof's syndrome
D81.5Purine nucleoside phosphorylase [PNP] deficiency
D81.6Major histocompatibility complex class I deficiency
D81.7Major histocompatibility complex class II deficiency
D81.810Biotinidase deficiency
D81.818Other biotin-dependent carboxylase deficiency
D81.819Biotin-dependent carboxylase deficiency, unspecified
D81.82Activated Phosphoinositide 3-kinase Delta Syndrome [APDS]
D81.89Other combined immunodeficiencies
D81.9Combined immunodeficiency, unspecified

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