D81.6
Major histocompatibility complex class I deficiency
Billable FY2026 2025-10-01 → 2026-09-30
Exclusions 13 never code together
D80-D89block
D50-D89chapter
Excludes2
- autoimmune disease (systemic) NOS (M35.9)M35.9
- certain conditions originating in the perinatal period (P00-P96)P00-P96
- complications of pregnancy, childbirth and the puerperium (O00-O9A)O00-O9A
- congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)Q00-Q99
- endocrine, nutritional and metabolic diseases (E00-E88)E00-E88
- human immunodeficiency virus [HIV] disease (B20)B20
- injury, poisoning and certain other consequences of external causes (S00-T88)S00-T88
- neoplasms (C00-D49)C00-D49
- symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)R00-R94
Excludes1 — never code together. The excluded condition and this one are mutually exclusive.
Excludes2 — not included here, but the patient may have both. Code both when documented.
Instructional notes 4
Index entries leading here 2
Alongside this code 9 same parent — D81
D81.0 Severe combined immunodeficiency with reticular dysgenesis D81.1 Severe combined immunodeficiency w low T- and B-cell numbers D81.2 Severe combined immunodef w low or normal B-cell numbers D81.3 Adenosine deaminase [ADA] deficiency category D81.4 Nezelof's syndrome D81.5 Purine nucleoside phosphorylase [PNP] deficiency D81.7 Major histocompatibility complex class II deficiency D81.8 Other combined immunodeficiencies category D81.9 Combined immunodeficiency, unspecified
Additional references
AHA Coding Clinic® Licence required
Official coding advice for this code. Published by the American Hospital Association. Not included in this installation.