ICD-10 Code D68.1 – Hereditary factor XI deficiency (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Hereditary factor XI deficiency is D68.1.
2026 ICD-10-CM Diagnosis Code D68.1 – Hereditary factor XI deficiency

What it is

D68.1 identifies an inherited bleeding disorder caused by reduced or absent factor XI, a clotting protein. It is used when documentation specifies hereditary factor XI deficiency.

Clinical signs

Clinical features vary; refer to documentation. Patients may have a bleeding tendency, often noticed after surgery, dental work, or trauma, rather than spontaneous major bleeding.

When to use this code

Use D68.1 when the record documents hereditary factor XI deficiency, factor XI deficiency, or hemophilia C. Apply it for confirmed congenital cases, including encounters for evaluation, monitoring, or pre-procedure planning.

Code the condition when the provider links bleeding risk to the inherited deficiency. Check documentation if the note only mentions an abnormal coagulation test without a stated diagnosis.

Do not use for

Do not use this code for acquired coagulation disorders, nonspecific bleeding, or other factor deficiencies. Check documentation if the diagnosis is uncertain or not clearly hereditary.

Coding tip

Look for explicit wording such as “hereditary factor XI deficiency” or “hemophilia C” before assigning D68.1.

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