Active Ingredient: Contains 600 International Units (IU) of human Coagulation Factor IX, also known as Christmas factor.
Formulation: It is a lyophilized (freeze-dried) powder for reconstitution, which must be mixed with a specified diluent (e.g., sterile water for injection) before use.
Source: The product can be either derived from human plasma (plasma-derived) or produced using recombinant DNA technology (recombinant Factor IX).
Concentration: After reconstitution, the final concentration is typically 120 IU/mL, making it a highly potent solution for intravenous administration.
Advantages
Specific Hemophilia B Treatment: Provides a precise and effective way to replace the missing or deficient Factor IX, which is the underlying cause of hemophilia B, to control and prevent bleeding.
Prophylaxis: Can be used for long-term prophylaxis against bleeding episodes in patients with severe hemophilia B, significantly improving their quality of life and reducing joint damage.
On-Demand Bleeding Control: Offers a rapid and reliable method to treat acute bleeding episodes in patients with Factor IX deficiency.1
Dose-Dependent Effect: The dose can be tailored based on the patient’s body weight and the severity of the bleeding or surgical procedure, allowing for individualized therapy.
Uses
Hemophilia B: The primary and most common indication is for the control and prevention of bleeding in patients with congenital Factor IX deficiency (Hemophilia B), also known as Christmas disease.2
Prophylaxis: Indicated for routine prophylaxis in hemophilia B to prevent recurrent bleeding episodes, particularly into joints and muscles.
Surgical Management: Used to manage perioperative bleeding in hemophilia B patients undergoing surgery or dental procedures, ensuring adequate hemostasis.
Acquired Factor IX Deficiency: Can be used to control bleeding in patients with an acquired Factor IX deficiency, although this is a less common indication.
Nature
Intrinsic Coagulation Pathway: Coagulation Factor IX is a vitamin K-dependent protein that plays a crucial role in the intrinsic pathway of the blood coagulation cascade.3
Zymogen Activation: It circulates as an inactive zymogen.4 Upon activation, it becomes Factor IXa, a serine protease that, in a complex with Factor VIIIa, activates Factor X.5
Mechanism of Action: By replacing the deficient Factor IX, the administered product restores a critical step in the coagulation cascade. This allows for the proper and timely formation of fibrin, which stabilizes a clot and stops bleeding.
Risk of Inhibitors: A significant characteristic of Factor IX products is the potential for the patient’s immune system to develop neutralizing antibodies, known as inhibitors, which can render the product ineffective.
Storage
Unreconstituted Powder: The powder for injection should be stored refrigerated at a temperature of 2∘C to 8∘C (36∘F to 46∘F).
Protection from Light: It must be kept in its original packaging, protected from light, to maintain its stability.
Reconstitution & Immediate Use: The product must be reconstituted with the provided sterile diluent immediately before use. The reconstituted solution should be administered within a few hours (typically within 4 hours) to maintain the activity of the factor.6
No Freezing/Refrigeration Post-Reconstitution: The reconstituted solution should not be frozen or refrigerated. Any unused portion must be discarded safely as the product does not contain preservatives.
