Factor X Deficiency Treatment & Management

Updated: Mar 01, 2017
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Perumal Thiagarajan, MD  more...
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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.

Therapeutic measures may include the following:

  • Factor X, human (Coagadex) can be initiated with 25 IU/kg IV for on-demand treatment at the first sign of a bleeding episode. Calculation of desired in vivo peak increase in factor X is required for precise dose. It may also be used for mild hereditary factor X deficiency to decrease bleeding risk during and after surgical procedures. [45]
  • A high-purity, plasma-derived, safe and effective factor X concentrate has been recommended for patients with hereditary factor X deficiency. [46]
  • Infusion of fresh frozen plasma (Octaplas) 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. Perioperative prothrombin complex concentrate has also been employed to treat acquired factor X deficiency associated with systemic light-chain amyloidosis. [47]
  • 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.

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Surgical Care

For patients with acquired factor X deficiency due to amyloidosis, splenectomy has proven beneficial in restoring circulating factor X levels. [48, 49] This presumably occurs via the debulking of splenic amyloid.

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Consultations

Consultation in patients with factor X deficiency include hematologists and, in cases of congenital factor X deficiency, genetic counselors.

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Diet and Activity

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

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.

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