D68.1

Billable

Hereditary factor XI deficiency

HCC Category Mapping

V28HCC 112 — Von Willebrand Disease and Other Coagulation Defects

0.247

V24HCC 48 — Coagulation Defects and Other Specified Hematological Disorders

0.209

ESRDHCC 48 — Coagulation Defects and Other Specified Hematological Disorders

0.000

What This Code Means

This is a rare inherited bleeding disorder where the body doesn't produce enough of a protein called factor XI, which is needed for blood to clot properly. People with this condition may experience excessive bleeding after injuries or surgery.

Coding Tips

•Verify the diagnosis is specifically hereditary factor XI deficiency and not acquired factor XI deficiency (D68.4), as the distinction affects coding accuracy
•Document whether the patient has a personal or family history of this condition, as this hereditary disorder often requires genetic counseling documentation

Clinical Significance

Hereditary factor XI deficiency (Hemophilia C) is an autosomal bleeding disorder most commonly seen in Ashkenazi Jewish populations, with a prevalence of 1 in 450 in that group. Unlike Hemophilia A and B, bleeding severity does not correlate well with factor XI levels, and patients may be asymptomatic until challenged by surgery or trauma. Spontaneous bleeding is uncommon.

Documentation Requirements

✓Document factor XI activity level, bleeding history (particularly post-surgical or post-traumatic), and ethnic background when relevant.
✓Record whether the patient is heterozygous (partial deficiency) or homozygous/compound heterozygous (severe deficiency).
✓Note treatment history including fresh frozen plasma or factor XI concentrate use.

Commonly Confused Codes

D66 (Hemophilia A) and D67 (Hemophilia B) — more severe inherited bleeding disorders with different factor deficiencies D68.2 (Hereditary deficiency of other clotting factors) — captures other rare factor deficiencies beyond VIII, IX, and XI D68.311 (Acquired hemophilia) — autoimmune condition rather than inherited.

Code Hierarchy

└D68Other coagulation defects └D68.1Hereditary factor XI deficiency

└D68.1Hereditary factor XI deficiency