ICD-10 Code for Agammaglobulinemia
Also indexed as (acquired (secondary)) (nonfamilial)
The ICD-10-CM code for agammaglobulinemia is D80.1 — Nonfamilial hypogammaglobulinemia. This is the code the Alphabetic Index gives for agammaglobulinemia with no further qualifier, valid for FY2027 (October 1, 2026 – September 30, 2027).
Which agammaglobulinemia code should you use?
The Alphabetic Index lists 3 codes under Agammaglobulinemia. Which one applies depends on the qualifier documented in the record — the type, site, cause, or associated condition. Find the qualifier below, then verify the code in the Tabular List before using it.
By type
| autosomal recessive | D80.0 | Hereditary hypogammaglobulinemia |
| Bruton's X-linked | D80.0 | Hereditary hypogammaglobulinemia |
| common variable | D80.1 | Nonfamilial hypogammaglobulinemia |
| congenital sex-linked | D80.0 | Hereditary hypogammaglobulinemia |
| hereditary | D80.0 | Hereditary hypogammaglobulinemia |
| lymphopenic | D81.9 | Combined immunodeficiency, unspecified |
| Swiss type | D80.0 | Hereditary hypogammaglobulinemia |
| X-linked | D80.0 | Hereditary hypogammaglobulinemia |
Index terms and codes are reproduced from the official ICD-10-CM FY2027 release. The Alphabetic Index is a finding aid — a code must always be verified in the Tabular List before it is reported. Data sources