Factor VII Deficiency Treatment & Management

Updated: Sep 12, 2020
  • Author: Lucas Barreira Angelim, MD; Chief Editor: Perumal Thiagarajan, MD  more...
  • Print
Treatment

Medical Care

Management of bleeding in patients with factor VII (FVII) deficiency varies according to the site and severity of bleeding and the patient's baseline FVII activity level. Mild bleeding (eg, bruising and skin lacerations) can usually be controlled by applying local pressure at the bleeding site. Minimal mucosal bleeding episodes, such as epistaxis and during dental procedures, can be managed with antifibrinolytic agents or fibrin glue.

More severe bleeding may require FVII/FVIIa replacement. Current options for FVII replacement therapy include the following:

  • Plasma-derived FVII (pdFVII)
  • Recombinant activated FVII (rFVIIa)
  • Fresh frozen plasma (FFP)
  • Prothrombin complex concentrate (PCC)

For spontaneous hemorrhage or mild trauma, therapeutic FVII levels of 5-10% are sufficient to stop bleeding. This level may be achieved by administering fresh frozen plasma (FFP) at a dose of 5-10 mL/kg of body weight and repeating the dose every 8-12 hours for 1-2 days.

For major hemorrhage or surgery, FFP may be administered in a loading dose of 15-20 mL/kg and followed by 3-6 mL/kg every 8-12 hours until the surgical wound heals. This may require 5-7 days of treatment. Potential drawbacks of plasma infusion include volume overload, infectious complications, or an inability to achieve high levels of FVII.

Highly purified FVII concentrates are useful in patients with severe bleeding or as prophylaxis for surgery. Unlike factor VIII and factor IX deficiencies, for which levels of 100% are required before surgery, FVII deficiency requires levels in the range of 10-15% to produce efficient hemostasis. For major surgery, trough levels of FVII must not fall to less than 20 U/dL.

PCCs offer a high concentration of FVII but contain varying amounts of unwanted vitamin K–dependent coagulation factors, thus presenting an increased risk of thromboembolic complications. When PCCs are used, doses of 50 U/kg every 8 hours for 24 hours, followed by plasma infusions, have been shown to be effective for major orthopedic surgery.

The US Food and Drug Administration (FDA) has approved rFVIIa for the prevention and treatment of bleeding episodes in patients with congenital FVII deficiency. Because rFVIIa is produced by recombinant technology, it does not carry the risk of infectious complications. The recommended dose of rFVIIa for treatment of hemorrhage is 15–30 mcg/kg every 4–6 h until hemostasis is achieved. For perioperative replacement therapy, a dose of approximately 20 mcg/kg has proved effective in over 95% of cases; the  infusion should be repeated approximately 8 times in patients at high risk (ie, those with a history of major bleeding). [21]

Although rFVIIa is approved for bolus infusion only, continuous infusion of clotting factor concentrates is recommended for patients with hemophilia, as it avoids peaks and troughs in factor levels; may reduce the amount of clotting factor concentrates administered and, consequently, cost; and reduces the administative burden. A review by Rajpurkar and Cooper identified studies and case reports of continuous rFVIIa infusion for 46 surgeries (major and minor) in 26 patients with congenital FVII deficiency, and concluded that the practice is effective and safe. Perioperative dosing ranged from 0.2–30.0 mcg/kg/h, with a duration of treatment ranging from 8 hours to 10 days. [22]

Patients with severe congenital factor VII deficiency who have experienced central nervous system or gastrointestinal bleeding or hemarthrosis are at risk for further major or life-threatening hemorrhage and may be considered for long-term prophylaxis. In the literature, dosages for prophylaxis have varied, with 20-30 mcg/kg of rFVIIa twice or three times weekly reported as effective. Dose and frequency can be tailored on the basis of clinical response, as neither FVII activity nor thrombin generation parameters predict the efficacy of prophylaxis. [23, 24]

Next:

Surgical Care

Prophylactic administration of factor VII concentrates or plasma may help minimize bleeding during surgery. For surgery, factor VII concentrates have been used in doses ranging from 8-40 U/kg every 4-6 hours.

Fibrin glue or fibrin tissue adhesives have been used as adjunctive therapy to achieve hemostasis. Fibrin glue contains fibrinogen, thrombin, and factor XIII.

Antifibrinolytic agents such as epsilon-aminocaproic acid and tranexamic acid have been used to enhance hemostasis during dental procedures as an adjunct to replacement therapy.

Previous
Next:

Consultations

Obtain consultations with the following, as needed:

  • Orthopedist
  • Physical therapist
  • General surgeon
  • Dental surgeon
  • Genetic counselor
Previous
Next:

Diet and Activity

Instruct patients to maintain a regular, healthy diet without restrictions.

Recommend that patients limit activity of the involved joints or muscles during acute bleeding episodes. Early physical therapy is recommended once bleeding is resolved to prevent contractures or deformity.

Previous
Next:

Complications

Infectious complications from transfusion of plasma or factor concentrates include HIV infection and hepatitis. Transmission of other infectious disease (eg, variant Creutzfeldt-Jakob disease) is always a theoretical possibility. Recurrent hemarthrosis leads to joint deformities and disability. Occupational and social rehabilitation is necessary.

Previous
Next:

Long-Term Monitoring

Perform annual history and physical examinations and test for hepatitis virus and HIV. Order MRI or CT scans for follow-up of joint deformities. Regular screening tests include cholesterol, prostate-specific antigen levels, colonoscopy, and mammography. In addition, vaccination for hepatitis A and B viruses, dental care, orthopedic follow-up, and patient education are recommended.

Previous