Indications: Treatment and prophylaxis of bleeding in patients with haemophilia B (congenital factor IX deficiency). BeneFIX can be used for all age groups.¹
 

Dosing: Dose and duration of the substitution therapy depend on the severity of the factor IX deficiency, on the location and extent of bleeding, and on the patient’s clinical condition. The number of units of factor IX administered is expressed in IU.¹
 

On demand treatment: The calculation of the required dose of BeneFIX can be based on the finding that one unit of factor IX activity per kg body weight is expected to increase the circulating level of factor IX, an average of 0.8 IU/dL (range from 0.4 to 1.4 IU/dL) in patients ≥ 12 years.¹
 

Prophylaxis: BeneFIX may be administered for long term prophylaxis against bleeding in patients with severe haemophilia B. In a clinical study for routine secondary prophylaxis the average dose for PTPs was 40 IU/kg (range 13 to 78 IU/kg) at intervals of 3 to 4 days.¹
 

Contraindications: Hypersensitivity to the active substance or to any of the excipients. Known allergic reaction to hamster proteins.¹
 

Special warnings and precautions for use: Allergic-type hypersensitivity reactions are possible with BeneFIX. In patients with existing cardiovascular risk factors, substitution therapy with FIX may increase the cardiovascular risk. Inhibitors are an uncommon event in PTPs receiving factor IX-containing products. Because of the potential risk of thrombotic complications, clinical surveillance for early signs of thrombotic and consumptive coagulopathy should be initiated with appropriate biological testing when administering this product to patients with liver disease, to patients post-operatively, to new-born infants, or to patients at risk of thrombotic phenomena or DIC.¹
 

Traceability: In order to improve the traceability of biological medicinal products, the name and the batch number of the administered product should be clearly recorded. Patients can affix one of the peel-off labels found on the vial to document the batch number in their diary or for reporting any side effects.¹
 

Special populations: Sufficient data have not been obtained from clinical studies on the treatment of previously untreated patients (PUPs) with BeneFIX.¹
 

Fertility, pregnancy and lactation: Animal reproduction studies have not been conducted with factor IX. Based on the rare occurrence of haemophilia B in women, experience regarding the use of factor IX during pregnancy and breastfeeding is not available. Therefore, factor IX should be used during pregnancy and breast-feeding only if clearly indicated. The effect of BeneFIX on fertility has not been established.¹
 

Effects on ability to drive and use machines: BeneFIX has no influence on the ability to drive or use machines.¹
 

Adverse reactions: Hypersensitivity or allergic reactions (which may include angioedema, burning and stinging at the infusion site, chills, flushing, generalised urticaria, headache, hives, hypotension, lethargy, nausea, restlessness, tachycardia, tightness of the chest, tingling, vomiting, wheezing) have been observed and may in some cases progress  to severe anaphylaxis (including shock).Patients with haemophilia B may develop neutralising antibodies (inhibitors) to factor IX. If such inhibitors occur, the condition will manifest itself as an insufficient clinical response. There is a potential risk of thromboembolic episodes following the administration of factor IX products.¹
 

Very Common (≥ 1/10): Headache, cough and pyrexia¹
 

Common (≥ 1/100 to < 1/10): Hypersensitivity, dizziness, dysgeusia, phlebitis, flushing, vomiting, nausea, rash, urticaria, chest discomfort, infusion-site reaction and infusion-site pain.¹
 

DIC: disseminated intravascular coagulation; FIX: factor IX; IU: international unit; PTP: previously treated patient.

Reference:

1. Benefix Egyptian Drug Authority leaflet approval date: 14/11/2021. Revision Date: November 2020.