From the Guidelines
Fresh Frozen Plasma (FFP) is not the preferred treatment for Hemophilia A due to its insufficient Factor VIII levels, and its use should be limited to emergency situations when Factor VIII concentrates are unavailable. When considering the use of FFP in Hemophilia A, it's crucial to weigh the risks and benefits, particularly given the concerns about allergic reactions, pathogen inactivation, and transfusion-related acute lung injury, as highlighted in studies such as 1. The primary issue with using FFP for Hemophilia A is its low concentration of Factor VIII, which is essential for effective hemostasis.
- FFP contains approximately 1 unit of Factor VIII per mL, making it an inefficient choice for raising Factor VIII levels to the desired range for managing bleeding.
- For minor bleeding, Factor VIII levels should ideally be raised to 30-50%, and for major bleeding or surgery, levels should be 50-100%, which is challenging to achieve with FFP alone.
- The use of FFP also carries significant risks, including volume overload and allergic reactions, as noted in 1. If FFP must be used, a typical order might be: "Transfuse Fresh Frozen Plasma 15-20 mL/kg IV over 1-2 hours. Repeat as needed based on clinical response and coagulation parameters," but this should be approached with caution and only in the absence of more appropriate treatments like Factor VIII concentrates. Given the information from 1 regarding the management of factor deficiencies and the concerns associated with plasma products, the preference should always be for Factor VIII concentrates, which offer more precise dosing, minimal volume, and reduced risk of adverse effects compared to FFP.
From the Research
Sample Transfusion Order for Fresh Frozen Plasma in Hemophilia A
- The transfusion order for Fresh Frozen Plasma (FFP) in Hemophilia A should be based on the patient's weight and the desired increase in factor VIII levels 2.
- A dose of 12.4 ml/kg of FFP or Fresh Dry Plasma (FDP) has been used to effectively control bleeding episodes in patients with Hemophilia A 2.
- The guidelines for the use of FFP recommend its use to replace single coagulation factor deficiencies, where a specific or combined factor concentrate is unavailable, such as in Hemophilia A 3.
- FFP should only be used to treat bleeding episodes or prepare patients for surgery in certain defined situations, and its use should be justified by the presence of bleeding and disturbed coagulation 3.
Factor VIII Levels and Recovery
- The increment of factor VIII levels after FFP transfusion has been reported to be around 12.1 +/- 3.7% at 30 minutes after transfusion, with a recovery rate of 65.3 +/- 22.7% 2.
- The factor VIII levels have been shown to decrease to 78.9 +/- 12.3%, 55.6 +/- 13%, and 16.3% of the initial level at 2,8, and 24 hours after FDP transfusion, respectively 2.
- The use of recombinant factor VIII concentrates with enhanced half-life has been shown to improve the management of Hemophilia A, allowing for higher protection from bleeding and improved safety for physical activity 4.
Emerging Evidence and Role of Factor VIII
- Factor VIII has a long-established role in the management of Hemophilia A, but emerging evidence suggests that it may have roles beyond haemostasis, including in the cardiovascular system, angiogenesis, and maintenance of bone health 5.
- Supraphysiological factor VIII levels have been identified as a risk factor for venous thromboembolism, highlighting the need for careful management of factor VIII levels in patients with Hemophilia A 5.