How is acute bleeding treated in patients with pediatric factor VII deficiency?

Updated: Jun 22, 2021
  • Author: Helge Dirk Hartung, MD; Chief Editor: Hassan M Yaish, MD  more...
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Answer

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 factor VII 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 factor VII 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 factor VII. These concentrates have undergone viral attenuation during manufacturing. Determining the appropriate dosage for treatment of factor VII deficiency can be difficult. These agents carry a risk of thrombogenic complications, particularly with repeated administration.

  • Factor VII concentrates are purified plasma–derived preparations that have undergone a vapor-heat viral-inactivation process. If available, factor VII concentrates are preferred over untreated plasma. [7] 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 factor VII (rFVIIa) was originally developed to treat patients with hemophilia and inhibitors, but it can be used at lower doses for patients with congenital factor VII deficiency. With increasing experience and evaluation of rFVIIa for treatment and prophylaxis in factor VII deficiency, the benefits and safety profile in this setting are becoming clearer. However, venous and arterial thromboses have been reported via postmarketing sources. [8]

A literature review by Ramezanpour et al of patients with congenital factor VII deficiency found that of the 380 individuals in the study, 7% developed a factor VII inhibitor. Of these 27 inhibitor patients, severe factor VII deficiency was noted in the 26 with available coagulation levels (coagulation activity <10%). About 58% of those 26 patients experienced intracerebral hemorrhage, with the rate for non-inhibitor patients being below 10%. The presence of an inhibitor represents a challenge to replacement therapy in patients with factor VII deficiency. [9]


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