eMedicine Specialties > Pediatrics: General Medicine > Hematology
Factor VII Deficiency: Treatment & Medication
Updated: Dec 11, 2007
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
- Acute bleeds: Management of acute hemorrhage primarily consists of factor VII (FVII) replacement therapy to treat bleeding. Levels of more than 10% are usually hemostatic, although higher levels may be advisable in the event of a severe bleeding episode. Because FVII has a short half-life (3-4 h), repeat treatment may be necessary in all except minor bleeding episodes. Treatment alternatives include the following:
- Fresh frozen plasma is the least effective because of the volume required to provide adequate FVII replacement. No viral attenuation of this product means that a risk of viral transmission is present.
- Prothrombin complex concentrates contain factors II, IX, and X in addition to FVII. These concentrates have undergone viral attenuation during manufacturing. Determining the appropriate dosage for treatment of FVII deficiency can be difficult. These agents carry a risk of thrombogenic complications, particularly with repeated administration.
- FVII concentrates are purified plasma–derived preparations that have undergone a vapor-heat viral-inactivation process. If available, FVII concentrates are preferred over untreated plasma. When given at high doses, these concentrates carry a risk of thrombosis, likely because of other vitamin K-dependent factors that are present in significant concentrations.
- Recombinant activated FVII (rFVIIa) was originally developed to treat patients with hemophilia and inhibitors, but it can be used at lower doses for patients with congenital FVII deficiency. With increasing experience and evaluation of rFVIIa for treatment and prophylaxis in FVII deficiency, the benefits and safety profile in this setting are becoming clearer.
- Prophylaxis: The decision to embark on a program of prophylaxis is determined by the patient's clinical presentation and the number of clinically significant bleeding episodes requiring intervention. Consider prophylaxis for patients with recurrent hemarthrosis or intracranial hemorrhage. Beneficial results have been reported with regimens that vary from twice daily to twice weekly treatment.
Surgical Care
Maintaining FVII levels of at least 15-25% provides adequate hemostasis levels for most surgical procedures. Preoperative FVII replacement and monitoring of FVII levels is essential for major surgical interventions. Because of the short half-life (3-4 h), replacement therapy should continue postoperatively; the period of therapy is determined by the nature and extent of the procedure.
Consultations
- Consult a hematologist and/or hemostasis specialist for patients who require FVII replacement therapy.
- Genetic counseling and family studies are part of a complete evaluation.
Activity
In patients with severe FVII deficiency and a history of clinical bleeding, consider the risk of bleeding when choosing activities. Individuals should stay fit because good muscle strength protects joints. Patients are encouraged to avoid contact sports, wear appropriate protective gear, and choose activities, such as swimming, that promote muscle strength and flexibility with a low risk of joint injury.
Medication
Clotting factor concentrates
Clotting factor concentrates promote hemostasis by providing the deficient clotting factor to the coagulation cascade. Used for control and prevention of hemorrhagic episodes and surgical prophylaxis in patients with factor VII (FVII) deficiency.
Coagulation FVII, plasma-derived (FVII Concentrate)
Vitamin K–dependent glycoprotein that promotes hemostasis by activating extrinsic pathway of coagulation cascade. FVII concentrates, available from Baxter or Bio Products Laboratory (United Kingdom), are purified plasma–derived concentrates that have undergone viral inactivation with vapor heat.
Adult
10-50 IU/kg/dose IV; because of short half-life, repeat therapy may be required q6-12h to maintain hemostasis
Prophylaxis: 10-50 IU/kg 1-3 times/wk
Pediatric
Administer as in adults
None reported
Documented hypersensitivity; presence of inhibitory antibodies
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 FVII coagulant activity levels to evaluate response and recovery; monitor for signs of thrombosis or activation of coagulation system; thrombotic events are a risk in patients with crush injury, sepsis, or DIC
Coagulation FVIIa, recombinant (NovoSeven)
Activated FVII promotes hemostasis by activating the extrinsic pathway of coagulation cascade. Originally developed to treat patients with FVIII inhibitors. Doses lower than those recommended for patients with hemophilia are usually effective in patients with FVII deficiency.
Adult
15-30 mcg/kg/dose IV; because of short half-life, repeat therapy may be required q4-6h to maintain hemostasis after acute bleeding or for surgical procedures.
Pediatric
Administer as in adults
None reported
Documented hypersensitivity
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
FVII coagulant levels are difficult to interpret in the presence of infused FVIIa because standard assays are not designed for monitoring activated factors; monitoring for correction of PT may be more useful; monitor for signs of thrombosis or activation of coagulation system; thrombotic events are a risk in patients with crush injury, sepsis, or DIC
Antifibrinolytic agents
These agents are used to enhance hemostasis when fibrinolysis contributes to bleeding. They inhibit lysis of the fibrin clot and thus maintain hemostasis once achieved. Antifibrinolytics are particularly useful for bleeding from mucosal surfaces where fibrinolytic activity is high, such as the nose or oropharynx.
Aminocaproic acid (Amicar)
Lysine analogue that inhibits fibrinolysis by blocking binding of plasmin or plasminogen activators to lysine residues on fibrin.
Adult
30 g/d PO/IV in divided doses q3-6h; not to exceed 30 g/d; can be topically applied as a 10% solution in 0.9% NaCl
Pediatric
50-60 mg/kg/dose PO/IV q3-6h; not to exceed 18 g/m2/d; can be applied topically as a 10% solution in 0.9% NaCl
Coadministration with estrogens may increase clotting factors, leading to hypercoagulable state
Documented hypersensitivity; evidence of active intravascular clotting process; potentially fatal in DIC, differentiating between hyperfibrinolysis and DIC is important; bleeding from upper urinary tract (risk of clots being retained in ureter or bladder)
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
Caution in cardiac, hepatic, or renal disease
Tranexamic acid (Cyklokapron)
Alternative to aminocaproic acid. Lysine analogue that inhibits fibrinolysis by blocking binding of plasmin or plasminogen activators to lysine residues on fibrin.
Adult
25 mg/kg PO tid/qid or 10 mg/kg IV tid/qid if patient unable to take the PO dose; can be topically applied as a 10% solution in 0.9% NaCl
Pediatric
Administer as in adults
None reported
Documented hypersensitivity; evidence of DIC; bleeding from upper urinary tract (risk of clots being retained in ureter or bladder)
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
Caution in renal impairment
More on Factor VII Deficiency |
| Overview: Factor VII Deficiency |
| Differential Diagnoses & Workup: Factor VII Deficiency |
 Treatment & Medication: Factor VII Deficiency |
| Follow-up: Factor VII Deficiency |
| References |
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References
Mariani G, Herrmann FH, Dolce A, et al. Clinical phenotypes and factor VII genotype in congenital factor VII deficiency. Thromb Haemost. Mar 2005;93(3):481-7. [Medline].
Peyvandi F, Mannucci PM. Rare coagulation disorders. Thromb Haemost. Oct 1999;82(4):1207-14. [Medline].
Di Paola J, Nugent D, Young G. Current therapy for rare factor deficiencies. Haemophilia. Jan 2001;7 Suppl 1:16-22. [Medline].
Giansily-Blaizot M, Schved JF. Potential predictors of bleeding risk in inherited factorVII deficiency. Clinical, biological and molecular criteria. Thromb Haemost. Nov 2005;94(5):901-6. [Medline].
Mariani G, Dolce A, Marchetti G, Bernardi F. Clinical picture and management of congenital factor VII deficiency. Haemophilia. Oct 2004;10 Suppl 4:180-3. [Medline].
Mariani G, Lapecorella M, Dolce A. Steps towards an effective treatment strategy in congenital factor VII deficiency. Semin Hematol. Jan 2006;43(1 Suppl 1):S42-7. [Medline].
Perry DJ. Factor VII Deficiency. Br J Haematol. Sep 2002;118(3):689-700. [Medline].
Tuddenham EG, Pemberton S, Cooper DN. Inherited factor VII deficiency: genetics and molecular pathology. Thromb Haemost. Jul 1995;74(1):313-21. [Medline].
Further Reading
Keywords
inherited factor VII deficiency, FVII deficiency, vitamin K–dependent coagulation factors, hemorrhagic disorder, activated FVII, FVIIa, menorrhagia, hemarthrosis, thrombosis, epistaxis, anemia, hematoma
