ICD-10-CM D81: Combined immunodeficiencies

ICD-10-CM Diagnosis Code (2025)

Official Description for D81

Combined immunodeficiencies

Coding Notes & Guidelines for D81

Excludes1: Not Coded Here

autosomal recessive agammaglobulinemia (Swiss type) (D80.0)

Excludes1: Not Coded Here

biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)

Inclusion Terms

ADA deficiency NOS

Inclusion Terms
  • ADA deficiency with SCID
  • Adenosine deaminase [ADA] deficiency with severe combined immunodeficiency
Inclusion Terms
  • ADA2 deficiency
  • Adenosine deaminase deficiency type 2
Inclusion Terms
  • 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)
Inclusion Terms

Bare lymphocyte syndrome

Inclusion Terms

Multiple carboxylase deficiency

Inclusion Terms
  • Holocarboxylase synthetase deficiency
  • Other multiple carboxylase deficiency
Inclusion Terms

Multiple carboxylase deficiency, unspecified

Inclusion Terms

p110d-activating mutation causing senescent T cells, lymphadenopathy, and immunodeficiency [PASLI] disease

Inclusion Terms

Severe combined immunodeficiency disorder [SCID] NOS

Code Also
  • , if applicable, any associated manifestations, such as:
  • polyarteritis nodosa (M30.0)
  • stroke (I63.-)
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)

Child Codes (More Specific Codes for D81):

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 D81.31 - Severe combined immunodeficiency due to adenosine deaminase deficiency D81.32 - Adenosine deaminase 2 deficiency D81.39 - Other adenosine deaminase deficiency D81.4 - Nezelof's syndrome D81.5 - Purine nucleoside phosphorylase [PNP] deficiency D81.6 - Major histocompatibility complex class I deficiency D81.7 - Major histocompatibility complex class II deficiency D81.8 - Other combined immunodeficiencies D81.81 - Biotin-dependent carboxylase deficiency D81.810 - Biotinidase deficiency D81.818 - Other biotin-dependent carboxylase deficiency D81.819 - Biotin-dependent carboxylase deficiency, unspecified D81.82 - Activated Phosphoinositide 3-kinase Delta Syndrome [APDS] D81.89 - Other combined immunodeficiencies D81.9 - Combined immunodeficiency, unspecified

About ICD-10-CM Code D81 (Combined immunodeficiencies)

ICD-10-CM code D81 is a billable or specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2025 edition of ICD-10-CM D81 became effective on October 1, 2024.

This page provides detailed information about D81 - Combined immunodeficiencies. It includes common terminology, coding guidelines such as "Includes," "Excludes1," and "Excludes2" notes, and information on required 7th characters. Utilizing this information correctly is essential for accurate medical coding and claims submission for conditions like Combined immunodeficiencies.