D81

Combined immunodeficiencies

FY2027Chapter 3: Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism/D80–D89 Certain disorders involving the immune mechanism

Non-billable headerNot valid on its own. Use one of the more specific codes under it.

D81 is a non-billable ICD-10-CM code category for combined immunodeficiencies. Report a more specific code from it instead. It is part of the FY2027 code set, effective October 1, 2026. The more specific codes under it are 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 and 6 more. Its coding notes include 3 Excludes1 entries and 10 Excludes2 entries.

Excludes1

Not coded here. The listed conditions generally should not be reported together.

This code
  • autosomal recessive agammaglobulinemia (Swiss type) (D80.0)
Also from chapter/block level (2 notes)
Inherited from D80–D89 Certain disorders involving the immune mechanism
  • autoimmune disease (systemic) NOS (M35.9)
  • functional disorders of polymorphonuclear neutrophils (D71-)

Excludes2

Not included here. Both codes may be reported if the patient has both conditions.

Also from chapter/block level (10 notes)
Inherited from D80–D89 Certain disorders involving the immune mechanism
  • human immunodeficiency virus [HIV] disease (B20)
Inherited from Chapter 3 Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
  • autoimmune disease (systemic) NOS (M35.9)
  • certain conditions originating in the perinatal period (P00-P96)
  • complications of pregnancy, childbirth and the puerperium (O00-O9A)
  • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
  • endocrine, nutritional and metabolic diseases (E00-E88)
  • human immunodeficiency virus [HIV] disease (B20)
  • injury, poisoning and certain other consequences of external causes (S00-T88)
  • neoplasms (C00-D49)
  • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)

Includes

Further defines or gives examples of the content of the category.

Also from chapter/block level (3 notes)
Inherited from D80–D89 Certain disorders involving the immune mechanism
  • defects in the complement system
  • immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
  • sarcoidosis

More specific codes under D81 10