Hereditary Factor X Deficiency (HFXD) May Not Have Prolonged aPTT

Prevalence and PT/aPTT for congenital coagulation factor deficiencies^4,5,13,14

Factor X deficiency lab values vary across patients. Some patients may present with prolonged PT and prolonged aPTT, while other patients may present with prolonged PT and normal aPTT¹⁴

Factor Deficiency	Estimated Prevalence	PT	aPTT
Rare Bleeding Disorders
Factor II (prothrombin)	1 in 2 million	Prolonged	Normal or prolonged
Factor V	1 in 1 million	Prolonged	Prolonged
Factor VII	1 in 500,000	Prolonged	Normal
Factor X	1 in 500,000 to 1 in 1 million	Prolonged	Normal or prolonged
Combined Factors V/VIII	1 in 1 million	Prolonged	Prolonged
Factor XI	1 in 1 million	Normal	Prolonged
Factor XIII	1 in 2 million	Normal	Normal
More Common Bleeding Disorders
Factor VIII (Hemophilia A)	1 in 5,000 male births	Normal	Prolonged
Factor IX (Hemophilia B)	1 in 30,000 male births	Normal	Prolonged
von Willebrand Factor	1 in 100 to 1 in 10,000	Normal	Normal or prolonged

Consider testing for factor X deficiency if a patient with bleeding symptoms has a prolonged PT. A single blood test can confirm HFXD

aPTT, activated partial thromboplastin time; PT, prothrombin time.

Testing for HFXD

Early diagnosis of hereditary factor X deficiency may allow for earlier initiation of appropriate care and treatment

If hemophilia and von Willebrand disease have been excluded in a patient with bleeding symptoms—

The patient may have an underlying rare bleeding disorder, such as HFXD^4,5

Consider testing for HFXD if the patient’s prothrombin time (PT) is prolonged^4,5,7

Confirm factor X deficiency using a single test (CPT code 85260): plasma assay for Factor X Activity^7,15

For a patient with a lower-than-normal factor X assay result, use the factor X activity level to determine their Baseline Severity Classification^8,16

HFXD Baseline Severity Classification

Understanding HFXD baseline severity classification and how it differs from hemophilia^8,16-18

Factor X Baseline Severity Classification¹⁷

Determines bleeding risk based on baseline factor X activity (without treatment)

Based on analysis of registry data from the European Network of Rare Bleeding Disorders (EN-RBD) Group; included 45 patients with factor X deficiency out of 592 total patients with a rare bleeding disorder and a mean age of 31 years.^8,17

Chart showing bleeding risk ranges for Factor X Deficiency and Hemophilia A — **Baseline Severity Classification —** **Bleeding Risk Ranges for Factor X Deficiency and Hemophilia A^8,16-18**

Factor X severity classification ranges differ markedly from hemophilia^8,16-18

HFXD Clinical Bleeding Severity

Understanding HFXD clinical bleeding severity grades⁸

Factor X Clinical Bleeding Severity⁸

Assigns clinical bleeding grade based on documented bleeds*

From a cross-sectional study using EN-RBD data from 34 patients with HFXD.⁸

Chart showing Factor X activity and clinical bleeding severity — **Clinical Bleeding Severity Grades and Factor X Activity**⁷
(mean, 95% confidence interval [CI])

Grade III: Spontaneous major bleeding—hemarthrosis, CNS, GI, umbilical cord, intramuscular hematomas requiring hospitalization.

Grade II: Spontaneous minor bleeding—bruising, ecchymosis, minor wounds, oral cavity, epistaxis, menorrhagia.

Grade I: Bleeding after trauma or drug ingestion (antiplatelet or anticoagulant therapy).

Asymptomatic: No documented bleeding episodes.

Clinical bleeding severity strongly correlates with factor X activity level⁸

However, patients may vary in their bleed severity relative to factor X activity level, so ongoing monitoring for bleed occurrence is crucial⁸

*Bleeding episodes were classified into four bleeding severity categories relying on bleed location, potential clinical impact, and spontaneity; patients were assigned to a category if they had at least one documented bleeding episode matching the defined bleeding severity and no episode matching the higher severity grade. Linear regression analysis was performed, adjusting for age at data collection, sex, and center where diagnosis was made.

Learn More About How Hereditary Factor X Deficiency is Managed

References: 1. National Institutes of Health. Doherty T, et al. Bleeding disorders. StatPearls [Internet]. https://www.ncbi.nlm.nih.gov/books/NBK541050/. Updated April 3, 2023. Accessed August 24, 2023. 2. LearnHaem, Haematology made simple. 4 Nov. 2020. Accessed October 2, 2025. https://www.learnhaem.com/courses/coagulation/lessons/normal-haemostasis/topic/the-revised-coagulation-cascade/. 3. Tarantino MD. Haemophilia. 2021;27(4):531-543. doi: 10.1111/hae.14223. 4. Kamal AH, et al. Mayo Clin Proc. 2007;82(7):864-873. 5. Palla R, et al. Blood. 2015;125(13):2052-2061. 6. Menegatti M, Peyvandi F. Thromb Hemost. 2024 Aug 29. doi: 10.1055/s-0044-1789595. Epub ahead of print. 7. Peyvandi F, et al. Blood Reviews. 2021;50:100833. 8. Peyvandi F, Palla R, et al. J Thromb Haemost. 2012;10:615-621. 9. Branchford B, et al. Blood Coagul Fibrinolysis. 2024;35(3):73-81. 10. Herrmann FH, et al. Haemophilia. 2006;12:479-489. 11. Byams V, et al. J Women’s Health. 2022;31(3):301-309. 12. Shapiro A. Expert Opin Drug Metab Toxicol. 2017;13(1):97-104. 13. National Organization for Rare Disorders. Updated June 6, 2023. Accessed October 25, 2024. https://rarediseases.org/rare-diseases/factor-x-deficiency/. 14. Medline Plus. https://medlineplus.gov/genetics/condition/von-willebrand-disease/#synonyms. Updated August 8, 2023. Accessed November 1, 2024. 15. LabCorp. https://www.labcorp.com/tests/086306/factor-x-activity. Accessed November 5, 2024. 16. Peyvandi F, et al. Brit J Haematol. 1998;102:626-628. 17. Peyvandi F, et al. J Thromb Haemost. 2012;10:1938-1943. 18. Srivastava A, et al. Haemophilia. 2020;26(Suppl 6):1-158. doi: 10.1111/hae.14046. 19. Medical and Scientific Advisory Council (MASAC) of the National Bleeding Disorders Foundation. MASAC Document #290. https://www.bleeding.org/healthcare-professionals/guidelines-on-care/masac-documents/masac-document-290- masac-recommendations-concerning-products-licensed-for-the-treatment-of-hemophilia-and-selected-disorders-of-the-coagulation-system. Accessed March 31, 2025. 20. Escobar M, Kavakli K. Haemophilia. 2024;30:59-67.

Indications and Usage for COAGADEX

COAGADEX, a plasma-derived blood coagulation factor X concentrate, is indicated in adults and children with hereditary factor X deficiency for:

Routine prophylaxis to reduce the frequency of bleeding episodes
On-demand treatment and control of bleeding episodes
Perioperative management of bleeding in patients with mild, moderate and severe hereditary factor X deficiency

Contraindication for COAGADEX

COAGADEX is contraindicated in patients who have had life-threatening hypersensitivity reactions to COAGADEX.

Important Safety Information for COAGADEX

Allergic type hypersensitivity reactions, including anaphylaxis, are possible with COAGADEX. If symptoms occur, patients should discontinue use of the product immediately, contact their physician, and administer appropriate treatment.

The formation of neutralizing antibodies (inhibitors) to factor X is a possible complication in the management of individuals with factor X deficiency. Carefully monitor patients taking COAGADEX for the development of inhibitors by appropriate clinical observations and laboratory tests.

COAGADEX is made from human plasma and may contain infectious agents, e.g. viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent. No cases of transmission of viral diseases, vCJD or CJD, have been associated with the use of COAGADEX.

In clinical studies, the most common adverse reactions (frequency ≥5% of subjects) with COAGADEX were infusion site erythema, infusion site pain, fatigue and back pain.

Please see complete Prescribing Information for COAGADEX.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit https://www.fda.gov/medwatch, or call 1-800-FDA-1088.
You may also call Kedrion at 1-866-398-0825 or email US_Medicalinfo@kedrion.com.