2026 ICD-10-CM Diagnosis Code D68.1Hereditary factor XI deficiency

ICD-10-CM Codes›D50–D89›D65-D69›D68

ICD-10-CM D68.1
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

D68.1 is a billable ICD-10-CM diagnosis code for hereditary factor XI deficiency. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as factor XI deficiency. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Coagulation and hemorrhagic disorders.

Code Identity

ICD-10-CM Code
D68.1
Billable Status
Yes — Valid for Submission
Code Describes
Hereditary factor XI deficiency
Short Description
Hereditary factor XI deficiency
Same as the full description in the CMS dataset.
Parent Code
D68
Other coagulation defects

Code Classification

ChapterD50–D89Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
SectionD65-D69Coagulation defects, purpura and other hemorrhagic conditions
CategoryD68Other coagulation defects
This CodeD68.1Hereditary factor XI deficiency

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Factor XI deficiency
  • Factor XI deficiency, type I
  • Factor XI deficiency, type II
  • Factor XI deficiency, type III
  • Hereditary factor XI deficiency disease

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Hemophilia C
  • Plasma thromboplastin antecedent PTA deficiency
  • Rosenthal's disease

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Deficiency, deficient
      • anti-hemophilic
        • factor (A)
          • C
    • Deficiency, deficient
      • factor
        • XI (congenital) (hereditary)
    • Deficiency, deficient
      • plasma thromboplastin
        • antecedent (PTA)
    • Deficiency, deficient
      • PTA (plasma thromboplastin antecedent)
    • Disease, diseased
      • Rosenthal's (factor XI deficiency)
    • Hemophilia(classical) (familial) (hereditary)
      • C
    • Rosenthal's disease or syndrome

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR BLD006
Coagulation and hemorrhagic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Factor XI Deficiency

    a hereditary deficiency of blood coagulation factor xi (also known as plasma thromboplastin antecedent or pta or antihemophilic factor c) resulting in a systemic blood-clotting defect called hemophilia c or rosenthal's syndrome, that may resemble classical hemophilia.

  • Acquired Factor XI Deficiency

    an acquired coagulation disorder characterized by the partial or complete absence of factor xi activity in the blood.

  • Factor XI Deficiency

    a coagulation disorder characterized by the partial or complete absence of factor xi activity in the blood.

  • Hereditary Factor XI Deficiency|Hemophilia C|Hereditary Factor XI Deficiency Disease|Hereditary Factor XI Deficiency Disease|Hereditary factor XI deficiency

    a rare inherited bleeding disorder caused by deficiency of coagulation factor xi. it may be asymptomatic or manifest with bleeding.

Patient EducationClinical

Bleeding Disorders

Normally, if you get hurt, your body forms a blood clot to stop the bleeding. For blood to clot, your body needs cells called platelets and proteins known as clotting factors. If you have a bleeding disorder, you either do not have enough platelets or clotting factors or they don't work the way they should.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert D68.1 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
286.2 Cong factor xi disorder
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About D68.1Overview

Is D68.1 (Other coagulation defects) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report hereditary factor XI deficiency on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of D68.1?

Under the General Equivalence Mappings, hereditary factor XI deficiency converts to ICD-9-CM 286.2 (cong factor xi disorder). The mapping is a direct match.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.