Factor V Deficiency Treatment & Management

Updated: Mar 09, 2021
  • Author: Olga Kozyreva, MD; Chief Editor: Perumal Thiagarajan, MD  more...
  • Print
Treatment

Medical Care

Although a plasma-derived factor V concentrate is undergoing preliminary testing, [22]  no concentrates of factor V are commercially available. Instead, fresh plasma or fresh frozen plasma (FFP, Octaplas) infusions are used to correct the deficiency temporarily and should be given daily during a bleeding episode. The loading dose of FFP is 15-20 mL/kg and then 3-6 mL/kg daily. Subsequent dosages depend on monitoring the factor V level by obtaining peak and trough factor V level assays. The half-life ranges from 24-36 hours, with the aim being a factor V level of 25%. Fluid overload and viral transmission may be a complication of plasma therapy.

Alternatively, platelet transfusions are emerging as an alternative to FFP. Factor V stored within platelet alpha granules has greater procoagulant potential and is released locally at sites of vascular injury. [23]

Preoperative and postoperative care when patients with factor V deficiency require surgery include the following:

  • In pregnant women, considerable bleeding can occur at the time of delivery; however, the administration of FFP can properly control bleeding, with excellent fetal outcome.

  • The safe level of factor V for adequate surgical hemostasis is 25% of the activity of factor V in normal control plasma. [24]

  • Postoperatively, FFP should be administered for 3-10 days, with careful observation of wound bleeding.

  • Tooth extraction in a patient with factor V hereditary deficiency is safely performed with both supplementation of FFP and application of local hemostasis. [25]

Female patients with factor V deficiency can be given oral contraceptives to decrease menometrorrhagia, thereby improving anemia and decreasing transfusion needs. [17]

The optimal treatment of patients with factor V inhibitors is uncertain. Plasma exchange combined with immunosuppression has proved effective. [26] Fu et al were successful in using a combination of factor replacement, chemotherapy, and plasmapheresis in a patient with spontaneous, life-threatening intracranial bleeding caused by a factor V inhibitor. The patient deteriorated after initial treatment with FFP and platelet transfusions. He was subsequently treated with a combination of plasma exchange and chemotherapy, and he completely recovered. [27] The experience of Fu et al shows that combinations of therapies may be needed in patients with serious hemorrhage caused by acquired factor V deficiency.

Corticosteroids have been used successfully in acquired factor V deficiency. A case report by Wang et al describes successful elimination of a low-level factor V inhibitor and control of bleeding with corticosteroid therapy, in a patient with reduced factor V activity and a factor V inhibitor level of 1.9 BU, probably secondary to a urinary tract infection. [15] Gavva et al report two cases successfully treated with corticosteroids, one likely secondary to antibiotics and the other, to either hepatitis C virus or antibiotic exposure. [23] Wu et al report successful treatment with immunosuppressive therapy including steroids and cyclophosphamide in a patient with acquired factor V deficiency and life-threatening pelvic hemorrhage. [28]

Gene therapy is a promising future approach to treatment of factor V deficiency. Nakamura et al report successful correction of F5 mutation in induced pluripotent stem cells from a factor V–deficient patient through the use of a clustered regularly interspaced short palindromic repeats (CRISPR)/CRISPR-associated 9 (Cas9). [29]

Next:

Consultations

Consultations may include hematologists, blood bank specialists, pathologists, and others as indicated based on hemorrhagic complications.

Previous