ICD-10 Code D70.0 – Congenital agranulocytosis (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code D70.0 – Congenital agranulocytosis
What it is
D70.0 identifies congenital agranulocytosis, a rare condition present at birth that involves an abnormally low or absent granulocyte count. Use it when documentation specifically names this inherited or congenital form.
Clinical signs
Clinical features vary; refer to documentation. Typical findings include recurrent or severe bacterial infections, fever, mouth ulcers, and neutropenia noted on evaluation. Confirm that the record supports a congenital diagnosis.
When to use this code
Use D70.0 when the provider documents congenital agranulocytosis, congenital neutropenia with agranulocytosis, or a clearly congenital absence of granulocytes. Code it from birth records, genetics notes, or hematology documentation when the condition is identified as present since birth.
Do not use for
Do not use this code for acquired neutropenia, drug-induced agranulocytosis, or transient low white blood cell counts. Check documentation if the cause is unclear or if the condition is not stated as congenital.
Coding tip
When the chart says only “agranulocytosis,” verify whether it is congenital before assigning D70.0.