AFSTYLA 2000 IU Powder and Solvent for Solution for Injection

What is AFSTYLA 2000 IU

AFSTYLA 2000 IU is a recombinant human coagulation factor VIII used for the treatment and prevention of bleeding episodes in patients with hemophilia A (congenital factor VIII deficiency). It contains Lonoctocog alfa, a single-chain recombinant factor VIII produced using recombinant DNA technology in Chinese hamster ovary (CHO) cells.

• It is indicated in both adults and children with hemophilia A.
• AFSTYLA is not indicated for the treatment of von Willebrand disease.
• The product is supplied as a lyophilized powder with a solvent (Water for Injection - WFI) and a complete administration kit.
• Designed for intravenous use after reconstitution.

The product is engineered for enhanced stability and prolonged activity in the bloodstream compared to conventional factor VIII products, offering the potential for less frequent dosing in prophylaxis.

How to use AFSTYLA 2000 IU

AFSTYLA must be used under the supervision of a healthcare provider experienced in the treatment of hemophilia. Administration should be carried out following sterile technique and proper reconstitution procedures.

1. Bring the powder and solvent to room temperature before reconstitution.
2. Use the filter transfer device provided in the kit to mix the powder with the 2.5 ml WFI.
3. Gently swirl (do not shake) until the powder is fully dissolved, resulting in a clear or slightly opalescent solution.
4. Draw the reconstituted solution into the provided 5 ml syringe through the filter.
5. Inject intravenously at a rate not exceeding 10 ml per minute.

• Inspect the solution before use. Do not use if it contains particulate matter or is discolored.
• Do not mix AFSTYLA with other medicinal products or infusions in the same syringe or tubing.
• Dispose of all used equipment safely, following standard biohazard protocols.

Mode of Action AFSTYLA 2000 IU

Lonoctocog alfa, the active component in AFSTYLA, is a recombinant single-chain factor VIII molecule designed to have an increased affinity for von Willebrand factor (VWF). This enhanced binding improves its stability and prolongs its half-life.

• After intravenous administration, AFSTYLA temporarily replaces the missing or deficient factor VIII, restoring the coagulation cascade.
• It activates the intrinsic pathway of blood coagulation by acting as a cofactor for activated factor IX (FIXa), leading to the conversion of factor X to its activated form (FXa).
• FXa then converts prothrombin to thrombin, which in turn converts fibrinogen to fibrin, forming a stable blood clot.

By replenishing factor VIII levels, AFSTYLA enables the body to control and prevent bleeding episodes. Its single-chain structure contributes to a longer duration of activity, offering effective hemostasis with reduced dosing frequency compared to standard factor VIII products.

AFSTYLA 2000 IU Interactions AFSTYLA 2000 IU

AFSTYLA does not have any known pharmacological drug-drug interactions in the classical sense, as it is a biological replacement therapy. However, there are important considerations to be aware of in clinical practice:

• Anti-Factor VIII inhibitors: Patients may develop neutralizing antibodies (inhibitors) against factor VIII, which can reduce or nullify the effect of AFSTYLA.
• Laboratory assay interference: Some aPTT-based assays may give misleading results when measuring factor VIII activity in patients receiving AFSTYLA. Chromogenic substrate assays are recommended for accurate monitoring.
• Concomitant use with other coagulation factors: Caution is required when administering with other clotting agents or antifibrinolytics (e.g., tranexamic acid), as this may potentiate the risk of thrombosis.

Clinicians should monitor factor VIII activity levels during treatment and watch for reduced response or signs of inhibitor development, especially in previously untreated patients (PUPs).

Dosage of AFSTYLA 2000 IU

The dosage of AFSTYLA depends on the patient’s body weight, clinical condition, and whether it is being used for prophylaxis or treatment of bleeding episodes. Dose should always be individualized based on clinical response and factor VIII activity levels.

• On-demand treatment of bleeding: 20–50 IU/kg depending on severity and location of the bleed.
• Major surgery: Initial dose of 40–60 IU/kg, with maintenance doses every 8–24 hours to maintain desired factor VIII levels.
• Prophylaxis:
  • Children & adolescents: 15–50 IU/kg, 2–3 times per week.
  • Adults: 20–40 IU/kg, every 2–3 days or 2× weekly depending on bleeding phenotype.

The 2000 IU kit is typically suited for older children, adolescents, or adults depending on the calculated dose. Dose adjustments should be based on pharmacokinetic profiling when available.

Possible side effects of AFSTYLA 2000 IU

AFSTYLA is generally well tolerated; however, like all protein-based therapies, it may cause adverse effects. Most side effects are mild to moderate in severity.

• Common side effects:
  • Headache
  • Dizziness
  • Injection site reactions (e.g., pain, redness, swelling)
• Less common but serious effects:
  • Development of factor VIII inhibitors (neutralizing antibodies)
  • Hypersensitivity or allergic reactions (e.g., rash, itching)
  • Anaphylaxis (rare but potentially life-threatening)
• Laboratory abnormalities: In rare cases, abnormal clotting test results due to assay interference.

Patients should be advised to seek immediate medical attention if they experience signs of allergic reaction such as swelling of the face, difficulty breathing, or chest tightness.

AFSTYLA 2000 IU Contraindications AFSTYLA 2000 IU

AFSTYLA is contraindicated in patients with the following conditions:

• Known hypersensitivity to Lonoctocog alfa, to any of the excipients (e.g., mannitol, polysorbate 80), or to mouse/hamster proteins used in production.
• History of anaphylactic reaction to recombinant factor VIII products.

Caution is also advised in:

• Patients with a history of inhibitor formation (may lead to reduced efficacy).
• Pregnant and breastfeeding women (no adequate data; use only if clearly needed).

Each patient must be evaluated individually, and a thorough allergy history should be taken before initiating therapy.

Storage of AFSTYLA 2000 IU

Proper storage of AFSTYLA is essential to ensure its efficacy and safety. Follow these guidelines strictly:

• Store in a refrigerator at 2°C to 8°C (36°F to 46°F).
• Do not freeze. Freezing may damage the product or container.
• The product may be stored at room temperature (up to 25°C or 77°F) for a single period not exceeding 6 months. Once removed from refrigeration, do not return it to the refrigerator.
• Keep the vial in the original carton to protect from light.
• After reconstitution, the solution should be used immediately. If not used right away, it can be stored for up to 4 hours at room temperature. Do not refrigerate after reconstitution.

Discard any unused reconstituted solution and materials according to local regulations for medical waste.