ICD-10-CM Code D81

Combined immunodeficiencies

Version 2021 Non-Billable Code

Not Valid for Submission

D81 is a "header" nonspecific and non-billable code code, consider using a code with a higher level of specificity for a diagnosis of combined immunodeficiencies. The code is NOT valid for the year 2021 for the submission of HIPAA-covered transactions.

ICD-10:D81
Short Description:Combined immunodeficiencies
Long Description:Combined immunodeficiencies

Consider the following ICD-10 codes with a higher level of specificity:

  • 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
  • D81.89 - Other combined immunodeficiencies
  • D81.9 - Combined immunodeficiency, unspecified

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code D81:

Type 1 Excludes

Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • autosomal recessive agammaglobulinemia Swiss type D80.0

Clinical Information

  • SEVERE COMBINED IMMUNODEFICIENCY-. group of rare congenital disorders characterized by impairment of both humoral and cell mediated immunity leukopenia and low or absent antibody levels. it is inherited as an x linked or autosomal recessive defect. mutations occurring in many different genes cause human severe combined immunodeficiency scid.

Code Classification

  • Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50–D89)
    • Certain disorders involving the immune mechanism (D80-D89)
      • Combined immunodeficiencies (D81)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021

Information for Patients


Immune System and Disorders

Your immune system is a complex network of cells, tissues, and organs that work together to defend against germs. It helps your body to recognize these "foreign" invaders. Then its job is to keep them out, or if it can't, to find and destroy them.

If your immune system cannot do its job, the results can be serious. Disorders of the immune system include

  • Allergy and asthma - immune responses to substances that are usually not harmful
  • Immune deficiency diseases - disorders in which the immune system is missing one or more of its parts
  • Autoimmune diseases - diseases causing your immune system to attack your own body's cells and tissues by mistake

NIH: National Institute of Allergy and Infectious Diseases

  • Agammaglobulinemia (Medical Encyclopedia)
  • Aging changes in immunity (Medical Encyclopedia)
  • Chronic granulomatous disease (Medical Encyclopedia)
  • Graft-versus-host disease (Medical Encyclopedia)
  • Histiocytosis (Medical Encyclopedia)
  • Hyperimmunoglobulin E syndrome (Medical Encyclopedia)
  • Immune response (Medical Encyclopedia)
  • Immunodeficiency disorders (Medical Encyclopedia)
  • Selective deficiency of IgA (Medical Encyclopedia)

[Learn More]