eMedicine Specialties > Hematology > Coagulation, Hemostasis, and Disorders

Factor X: Treatment & Medication

Author: Christopher J Steen, MD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School
Coauthor(s): Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School; Pere Gascon, MD, PhD, Professor and Director, Division of Medical Oncology, Institute of Hematology and Medical Oncology, IDIBAPS, University of Barcelona Faculty of Medicine, Spain
Contributor Information and Disclosures

Updated: Dec 1, 2008

Treatment

Medical Care

Treatment of factor X deficiency is individualized for each patient. However, restoring circulating factor X levels to 10-40% of normal is usually adequate. Additionally, in patients with acquired factor X deficiency, treatment of the underlying cause may resolve the disorder.

  • Infusion of fresh frozen plasma is usually sufficient to treat most hemorrhagic episodes. A loading dose of 15-20 mL/kg intravenously is administered. Because of the relatively long half-life of factor X, the loading dose can be followed by maintenance doses of 3-6 mL/kg intravenously every 12-24 hours.
  • Prothrombin complex concentrates (PCCs) have also been used to increase factor X levels. PCCs contain factors II, VII, IX, and X and protein C. PCCs should be used cautiously to avoid factor X levels of more than 50% of normal, which can result in thromboembolic episodes.
  • Vitamin K administration may be useful in certain patients with acquired factor X deficiency; however, it has been amply demonstrated that patients with inherited factor X deficiency do not respond to vitamin K.
  • Cryoprecipitate does not contain factor X and is, therefore, ineffective in factor X deficiency.

Surgical Care

For patients with acquired factor X deficiency due to amyloidosis, splenectomy has proven beneficial in restoring circulating factor X levels.37,38 This presumably occurs via the debulking of splenic amyloid.

Consultations

  • Hematologists
  • Genetic counselors (in cases of congenital factor X deficiency)

Diet

No dietary restrictions are necessary in individuals with factor X deficiency. Patients are advised to decrease alcohol consumption to reduce the risk of liver disease.

Activity

Activity must be regulated based on the severity of the factor X deficiency and the presence or absence of symptoms. Because of the risk of hemorrhage following trauma, activities with high levels of physical contact are not recommended.

Medication

The goals of pharmacotherapy in those with factor X deficiency are to reduce morbidity and to prevent complications.

Blood-Product Derivatives

Blood-product derivatives are indicated for the correction of abnormal hemostatic parameters.


Fresh frozen plasma

Clotting factor X is contained in plasma, the fluid component of blood. Indications include bleeding in patients with congenital coagulation defects and multiple coagulation factor deficiencies (severe liver disease).

Adult

Loading dose: 10-20 mL/kg IV

Maintenance dose: 3-6 mL/kg IV q12-24h

Pediatric

Administer as in adults.

Pregnancy

A - Fetal risk not revealed in controlled studies in humans

Precautions

Viral contamination and infection are possible, but they are unlikely because of prescreening.


Prothrombin complex concentrate

Product made from pooled human plasma. Contains factors II, VII, IX, and X; protein C; and trace amounts of heparin to guard against thrombosis. The dose can be calculated depending on the concentration of protein C in a preparation. Preparations may vary.

Adult

Dosages must be clinically determined and are case dependent; the typical dosage is 50-125 U/kg; because of the risk of thrombotic complications, no more than 2-3 standard doses should be administered in the first 36-48 h.

Pediatric

Administer as in adults.

When administered simultaneously with recombinant FVIIa, may lead to thrombotic complications; should not be used in combination with antifibrinolytics, because this combination may increase the risk of thrombosis

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Monitor for thrombotic complications and hypersensitivity reactions; clinical response, PT, and aPTT should be closely monitored throughout the therapy; if there is no clinical response to the therapy with prothrombin complex concentrates, another treatment should be considered.

Vitamins, Fat-Soluble

Vitamin K is key cofactor in activating clotting factors in the coagulation cascade.


Phytonadione (AquaMEPHYTON)

Absorbed by the gut and stored in the liver. Necessary for the function of clotting factors in the coagulation cascade. Used to replace essential vitamins that are not obtained in sufficient quantities in the diet or to further supplement levels.

Adult

10 mg PO/IV/IM/SC single dose to replete liver stores

Pediatric

1 mg IM as single dose

Effects of warfarin sodium and dicumarol are antagonized by phytonadione; broad-spectrum antibiotics, quinidine, quinine, and salicylates may increase the phytonadione requirements.

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Ineffective in hereditary hypoprothrombinemia; the IV formulation has been associated with rare anaphylactoid reactions and death, even with careful, slow administration; transient flushing sensations and peculiarities of taste have been reported following vitamin K injection

More on Factor X

Overview: Factor X
Differential Diagnoses & Workup: Factor X
Treatment & Medication: Factor X
Follow-up: Factor X
References
Further Reading
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References

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  36. Herrmann FH, Navarette M, Salazar-Sanchez L, et al. Homozygous Factor X gene mutations Gly380Arg and Tyr163delAT are associated with perinatal intracranial hemorrhage. J Pediatr. Jan 2005;146(1):128-30. [Medline].

  37. Greipp PR, Kyle RA, Bowie EJ. Factor X deficiency in primary amyloidosis: resolution after splenectomy. N Engl J Med. Nov 8 1979;301(19):1050-1. [Medline].

  38. Rosenstein ED, Itzkowitz SH, Penziner AS, Cohen JI, Mornaghi RA. Resolution of factor X deficiency in primary amyloidosis following splenectomy. Arch Intern Med. Mar 1983;143(3):597-9. [Medline].

  39. McMahon C, Smith J, Goonan C, Byrne M, Smith OP. The role of primary prophylactic factor replacement therapy in children with severe factor X deficiency. Br J Haematol. Dec 2002;119(3):789-91. [Medline].

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Keywords

factor X, factor X deficiency, FX, FX deficiency, Stuart factor, Prower factor, Stuart-Prower factor, autoprothrombin III, thrombokinase, blood coagulation factor X, coagulation factor X, vitamin K deficiency, liver disease, hepatic disease, blood disorder, factor disorder, coagulation disorder, clotting disorder, bleeding disorder, blood factor deficiency, factor deficiency, easy bruising, hematuria, soft-tissue hemorrhages, hemarthroses, recurrent epistaxis, menorrhagia, congenital factor X deficiency, acquired factor X deficiency

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Contributor Information and Disclosures

Author

Christopher J Steen, MD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey Medical School
Christopher J Steen, MD is a member of the following medical societies: Alpha Omega Alpha and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Pere Gascon, MD, PhD, Professor and Director, Division of Medical Oncology, Institute of Hematology and Medical Oncology, IDIBAPS, University of Barcelona Faculty of Medicine, Spain
Pere Gascon, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, New York Academy of Medicine, New York Academy of Sciences, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Paul Schick, MD, Emeritus Professor, Department of Internal Medicine, Thomas Jefferson University Medical College; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital, Wynnewood, PA
Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Hematology, International Society on Thrombosis and Haemostasis, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center
Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Society of Hematology
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

CME Editor

Rebecca J Schmidt, DO, FACP, FASN, Professor of Medicine, Section Chief, Department of Medicine, Section of Nephrology, West Virginia University School of Medicine
Rebecca J Schmidt, DO, FACP, FASN is a member of the following medical societies: American College of Osteopathic Internists, American College of Physicians, American Medical Association, American Society of Nephrology, International Society of Nephrology, National Kidney Foundation, Renal Physicians Association, and West Virginia State Medical Association
Disclosure: Abbott Grant/research funds Speaking and teaching; Genzyme Honoraria Consulting; Amgen Honoraria Speaking and teaching; Ortho Biotech Honoraria Speaking and teaching

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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