From the Guidelines
Factor VII transfusion is recommended for patients with Factor VII deficiency when they are actively bleeding, preparing for surgery, or experiencing severe trauma, with a recommended dose of 15-30 µg kg-1 every 6 h until cessation of bleeding, and then every 12 h, as stated in the guidance from the SSC of the ISTH 1. When considering factor VII transfusion, it's crucial to assess the patient's individual situation, including the severity of deficiency, bleeding history, and current clinical condition.
- The standard treatment for factor VII deficiency involves the use of recombinant activated Factor VII (rFVIIa), which has been shown to be effective in managing perioperative bleeding in patients with congenital FVII deficiency, as well as in those with Glanzmann’s thrombasthenia and other inherited platelet disorders 1.
- The dosing of rFVIIa should be adjusted based on the patient's response to treatment, with a recommended dose of 15-30 µg kg-1 every 6 h until cessation of bleeding, and then every 12 h, as guided by the SSC of the ISTH 1.
- It's also important to note that rFVIIa requires certain conditions to be effective, including fibrinogen concentrations ≥1 g L-1, platelet counts ≥ 50 × 10^9 L-1, pH ≥ 7.2, and body temperature > 34 °C, as highlighted in the study 1.
- While the efficacy of rFVIIa for off-label indications remains unclear, the current data suggest that it may have a potential role in minimizing bleeding or blood product use during major hemorrhage management, although overall survival may not improve, as noted in the study 1.
From the Research
Factor 7 Deficiency Transfusion Recommendations
- Factor VII (FVII) deficiency is a rare coagulation disorder that can be treated with recombinant, activated FVII preparation or factor VII concentrate (human) immuno, vapor heated 2, 3.
- The decision to transfuse factors for factor 7 deficiency depends on the severity of the deficiency, the patient's bleeding history, and the presence of any bleeding complications 4, 5.
- Recombinant FVII concentrate is considered the best replacement therapy for FVII deficiency, and the dosing regimen may vary depending on the patient's condition and the specific product used 2, 4.
- For acute bleeding episodes, factor VII concentrate can be administered at doses ranging from 10-50 IU/kg, with the goal of achieving a peak factor VII level of approximately 100% 3.
- For surgical coverage, the peak factor VII level should be approximately 100%, with trough levels ranging from 8 to 65% over treatment periods of 24 hours to 16 days using treatment intervals of 6-12 hours 3.
- Long-term prophylaxis may be considered for patients with severe FVII deficiency, with doses of 10-50 IU/kg given one to three times a week 3, 4.
- It is essential to note that the correlation between FVII levels and bleeding phenotype is poor, and a personal and family history is crucial to determine the bleeding risk and plan for surgical and obstetrical prophylaxis 4, 5.
Treatment Options
- Recombinant activated factor VII (rFVIIa) and prothrombin complex concentrate (PCC) are also used off-label for bleeding in cardiac surgery that is refractory to conventional therapy 6.
- The choice between rFVIIa and PCC depends on the specific clinical situation and the patient's individual needs, with both options appearing to be safe and effective for the management of bleeding associated with cardiac surgery 6.