Hereditary Factor X Deficiency Is a Rare Bleeding Disorder that Affects About 1 in a Million People1,2

  • Characterized by insufficient levels of factor X
  • Affects both women and men
  • People with factor X deficiency have a high risk of excess bleeding
  • Often hereditary, as an autosomal recessive disorder
Person standing in the middle of the letter x
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Diagnostic Indicators of Factor X Deficiency Include Bleeding Symptoms and Prolonged PT and aPTT1,2

Diagram showing the steps to Factor X deficiency diagnosis including: 1. past history of symptoms, 2. ruling out other bleeding disorders, 3. testing and 4. diagnosis.
aPTT, activated partial thromboplastin time; PT, prothrombin time.
Early diagnosis of factor X deficiency may allow for earlier initiation of appropriate care and treatment

Factor X Deficiency May Present with Ongoing Symptoms

Photo representing Fernando
Fernando coped with more than 25 years of joint swelling, pain, and bruising, until he was tested and diagnosed with factor X deficiency after being hospitalized for an intracranial hemorrhage. (Actual patient experience)

Symptom occurrence with factor X deficiency (N=42)3

Red spot on wrist
Easy bruising
Red spot on wrist
Red spot on swollen elbow
Red spot on wrist


Important Note

Up to 1 in 5 symptomatic patients with factor X deficiency have had an intracranial hemorrhage.
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  • In this analysis, additional symptoms of factor X deficiency included hematomas (43%), gum bleeding (31%), gastrointestinal hemorrhage (12%), and hematuria (7%)
Patients with factor X deficiency may have a high risk of spontaneous, severe, or life-threatening bleeds3

The majority of women with factor X deficiency have a history of heavy menstrual bleeding3

Photo representing Isabelle
Isabelle struggled for years with heavy menstrual bleeding but was not tested or diagnosed with factor X deficiency until age 21, when she required a blood transfusion following dental surgery. (Actual patient experience)
Woman icon

In women:

  • Heavy menstrual bleeding occurs in up to 70% of women of reproductive age who have hereditary factor X deficiency3
Patients with factor X deficiency may present with bleeds related to women’s health

Undiagnosed and untreated neonates can be at high risk for severe bleeds

Affected neonates are at risk of experiencing devastating outcomes if hereditary factor X deficiency is not promptly recognized and treated

Newborn baby icon

Symptoms in neonates3,4:

  • Intracranial hemorrhage (ICH)
  • Gastrointestinal (GI) bleeding
  • Bleeding that won’t stop normally from the umbilical stump or post-circumcision
  • Abnormal bruising or bleeding

Diagnostic Indicator

Prolonged PT and aPTT is a diagnostic indicator that a test for factor X deficiency may be appropriate

Confirmatory Testing

Diagnosis is made via a plasma coagulation factor X activity assay

ICH and GI Bleeds are Likely to Occur Early in Life3,4

In a study of 42 adult and pediatric patients with symptomatic hereditary factor X deficiency, 21% (9 of 42) experienced ICH and 12% (5 of 42) experienced GI hemorrhage—

Calendar highlighting the 10th day with text: 9.7 days Median Age at ICH or GI Bleed
All study patients presenting with ICH (9/42) or GI bleed (5/42) were in the neonatal age group—and all ICH or GI bleeds presented within 27 days of birth (median 9.7 days)
CNS bleeds, particularly ICH, are the most common severe bleed type occurring in patients with factor X deficiency. The highest risk of ICH is within the first few months of life, when factor X activity is low.
“Patients with FXD often present in infancy with unprovoked umbilical stump, intracranial, or gastrointestinal bleeding…” 5
Zimowski KL, et al. 2020.
Use of plasma-derived factor X concentrate in neonates and infants with congenital factor X deficiency.
Bleeds due to factor X deficiency can result in permanent impairment or mortality in neonates

Severity Classification is Unique to Factor X 4,6

Severity Classification by Factor X Activity*

>40 IU/dL

icon showing a blood sample with less than a normal amount of factor X

Low bleeding risk

10–40 IU/dL

icon showing a blood sample with very little factor X

Minor spontaneous bleeding

<10 IU/dL

icon showing a blood sample with almost no factor X

High risk of major spontaneous bleeding

  • Decreasing factor X activity has been shown to correlate with increasing bleeding severity7
  • Analysis of registry data from the European Network of Rare Bleeding Disorders (EN-RBD) suggests maintaining a factor X level of ≥10 IU/dL as the appropriate target for prophylaxis, due to the high risk of major spontaneous bleeding (such as GI or CNS) at <10 IU/dL6,7 †
  • In the general population, factor X activity ranges from 65–120 IU/dL (reference ranges may vary)8

*Severity classification as shown is currently widely accepted but differs from that existing at the time of COAGADEX clinical trials, which classified “severe” factor X deficiency activity as <1 IU/dL.8

The COAGADEX prescribing information recommendation is to monitor trough blood levels of factor X targeting ≥5 IU/dL and adjust dosage to clinical response and trough levels.

Peyvandi F, et al. J Thromb Haemost. 2012.
A review of data by the Rare Bleeding Disorders Working Group under the Factor VIII and Factor IX Scientific and Standardisation Committee of the International Society on Thrombosis and Haemostasis, exploring the association between residual plasma coagulant factor activity and clinical bleeding profile for each rare bleeding disorder (RB; N=4,359 patients with RBDs in North American, European, UK, and Indian registries). Factor X severity classification based on analysis of registry data from the European Network of RBDs (EN-RBD; n=592 patients with RBDs).
Unexpected bleeding has been observed over a broad range of factor X deficiency4,6

Treatment Considerations

Clinical guidelines and expert consensus recommend use of plasma-derived factor X concentrate for the treatment of hereditary factor X deficiency4,7,9

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The 2022 National Hemophilia Foundation (NHF) MASAC treatment guidelines recommend only plasma-derived factor X concentrate for the treatment of hereditary factor X deficiency9
NHF Medical and Scientific Advisory Council (MASAC)
Recommendations concerning products licensed for the treatment of hemophilia and other bleeding disorders, 2022.
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“The treatment of factor X deficiency has evolved substantially, from plasma replacement therapy (fresh frozen plasma) to prothrombin complex concentrates (PCCs) to, most recently, the development and licensing approval of a high-purity, plasma-derived, specific single-factor concentrate (pdFX). While patient numbers are small, clinical studies have demonstrated the ability of pdFX therapy to improve hemostasis while limiting the safety concerns associated with earlier treatments such as volume overload (particularly in neonates and young children), anaphylaxis, and thrombosis.”7
Peyvandi F, et al.
Diagnosis, therapeutic advances, and key recommendations for the management of factor X deficiency.
Blood Reviews. 2021.
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“Single-factor FX concentrate is currently considered the standard of care for both on-demand and prophylactic treatment of patients with hereditary FX deficiency…The use of prophylaxis to reduce bleeding risk is essential for patients with FX deficiency given the risk of spontaneous and/or life-threatening bleeds.”4
Tarantino MD.
Occurrence and management of severe bleeding episodes in patients with hereditary factor X deficiency.
Haemophilia. 2021.
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“Patients with severe FX deficiency tend to be the most seriously affected patients with rare coagulation disorders…”10
Menegatti M, et al.
Treatment of rare factor deficiencies other than hemophilia.
Blood. 2019.
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References: 1. Brown DL, Kouides PA. Haemophilia. 2008;14(6):1176-1182. 2. Palla R, et al. Blood. 2015;125(13):2052-2061. 3. Hermann FH, et al. Haemophilia. 2006;12:479-489. 4. Tarantino MD. Haemophilia. 2021;27:531-543. 5. Zimowski KL, McGuinn YL, Abajas YL, et al. J Thromb Haemost. 2020;18:2551-2556. 6. Peyvandi F, Palla R, et al. J Thromb Haemost. 2012;10:615-621. 7. Peyvandi F, Auerswald G, Austin SK, et al. Blood Reviews. 2021;50:100833. 8. Peyvandi F, et al. Brit J Haematol. 1998;102:626-628. 9. Medical and Scientific Advisory Council (MASAC) of the National Hemophilia Foundation. MASAC Document #272. Accessed November 9, 2022. 10. Menegatti M, Peyvandi F. Blood. 2019;133:415-424.

Replace exactly what's missing

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 and moderate hereditary factor X deficiency

Limitation of Use

Perioperative management of bleeding in major surgery in patients with severe hereditary factor X deficiency has not been studied.

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.