ICD-10 Code D80.0 – Hereditary hypogammaglobulinemia (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Hereditary hypogammaglobulinemia is D80.0.
2026 ICD-10-CM Diagnosis Code D80.0 – Hereditary hypogammaglobulinemia

What it is

D80.0 identifies an inherited disorder marked by low immunoglobulin levels. It reflects a primary antibody deficiency that can leave the patient less able to fight infections.

Clinical signs

Patients often have recurrent bacterial infections, especially of the sinuses, ears, lungs, or gastrointestinal tract. Clinical features vary; refer to documentation for the specific immune findings and family history.

When to use this code

Use D80.0 when the record documents hereditary hypogammaglobulinemia or a clearly inherited hypogammaglobulinemia diagnosis. You may also use it when the clinician links recurrent infections to this primary antibody deficiency. Do not infer inheritance from low immunoglobulin levels alone.

Do not use for

Do not assign this code for acquired or secondary hypogammaglobulinemia, such as medication-related or disease-related immune suppression. Check documentation if the cause is not clearly hereditary.

Coding tip

Confirm that the provider documents hereditary cause and immune deficiency, not just abnormal lab results.

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