Generic drug of the therapeutic class: Haemostasis and blood
active ingredients: Factor IX
Powder and solvent for solution for injection
Box of 1 bottle of powder + 20 ml solvent bottle
Human coagulation factor IX is indicated for the treatment and prevention of hemorrhage in patients with hemophilia B (congenital factor IX deficiency).
Dosage BETAFACT 50 IU / mL Powder and solvent for solution for injection Box of 1 Bottle of powder + 20 ml solvent bottle
Treatment should be initiated under the supervision of a hemophilia specialist.
The dosage and duration of the substitution treatment depend on the severity of the factor IX deficiency, the location and extent of the bleeding event, and the clinical condition of the patient.
The number of units of factor IX administered is expressed in International Units (IU), calculated against the WHO standard for factor IX. The plasma activity of factor IX is expressed either as a percentage (relative to normal human plasma) or as International Units (relative to the international standard for plasma factor IX).
One IU of human coagulation factor IX corresponds to the amount of factor IX contained in one ml of normal human plasma.
The calculation of the required dose is based on the observation that one IU of human coagulation factor IX per kg of body weight increases the normal factor IX plasma activity by 1.08%. This dose is determined using the following formula:
Number of units to administer =
body weight (kg) desired increase in factor IX (%) (IU / dl) 0.93
The dose and frequency of injections should always be adapted to each case based on clinical effectiveness. Human coagulation factor IX should be infrequently injected more than once a day.
Plasma activity of factor IX should be maintained at rates and for a period dependent on bleeding events to be managed. The table below can be used to determine the dosage in the following hemorrhagic and surgical situations:
Intensity of bleeding
Type of surgical intervention
Factor IX plasma level to be achieved (%) (IU / dl)
Frequency of injections (hours)
Duration of treatment (days)
Premature haemarthrosis, muscle bleeding or oral cavity
Extended haemarthrosis, muscle bleeding or hematoma
Life-threatening haemorrhages such as cerebral hemorrhage, bleeding from the neck, major abdominal bleeding
20 to 40
30 to 60
60 to 100
Repeat every 24 hours, for at least 1 day, until the pain or bleeding accident stops
Repeat the injection every 24 hours for 3 to 4 days or more until discomfort and pain disappear
Repeat the injection every 8 to 24 hours until the risk of bleeding disappears
Minor of which tooth extraction
30 to 60
80 to 100
(pre and postoperative)
Every 24 hours for at least 1 day until the bleeding stops
Repeat the injection every 8 to 24 hours until wound healing, then continue treatment for at least 7 additional days to maintain the factor IX level between 30% and 60% (IU / dl)
Depending on the circumstances, doses larger than those calculated may be necessary, particularly during the initial injection.
During treatment, it is recommended to control the level of factor IX to adjust the dosage and frequency of injections. In the case of major surgery in particular, substitution treatment requires special monitoring with control of coagulation (plasma activity of factor IX). The response to human coagulation factor IX treatment, including in vivo recovery rates and observed half-lives, may vary between individuals.
In long-term prophylaxis in patients with severe hemophilia B, doses of 20 to 40 IU of human coagulation factor IX per kg of body weight should be administered every 3 to
4 days. In some cases, especially in young people, it may be necessary to increase the dosage or to shorten the interval between two injections.
The appearance of a factor IX inhibitor should be monitored. If the desired plasma factor IX level can not be achieved or the bleeding is not controlled after a proper dose, the presence of a factor IX inhibitor should be investigated by a bioassay.
If an inhibitor occurs at a rate of less than 10 Bethesda Units (BU) per ml, the increased dose of injected human coagulation factor IX may be sufficient to neutralize the inhibitor. In patients with an inhibitor level above 10 BU or a significant anamnestic response, the use of prothrombin complex concentrate (activated) or activated factor VII (FVIIa) should be considered. These treatments should be conducted under the supervision of a hemophilia specialist.
In clinical trials, 11 children under 6 years of age were treated with BETAFACT at a dose appropriate to their body weight, identical to that of adults (see Warnings and Precautions for Use "Special warnings and precautions" job ").
Method and route of administration
Reconstitute the preparation as described in section 6.6 "Special precautions for disposal and other handling". Inject intravenously.
It is recommended not to administer human coagulation factor IX at a rate greater than 4 ml / minute.
· Hypersensitivity to the active substance or to any of the excipients.
Betafact side effects
Hypersensitivity or allergic reactions (such as angioedema, burning and tingling at the injection site, chills, redness, generalized urticaria, headache, urticarial reactions, low blood pressure, lethargy, nausea, agitation, tachycardia, chest tightness, tingling), vomiting, asthmatic respiration) have been observed inconsistently in some patients treated with human coagulation factor IX preparations.
In some cases, these reactions have resulted in a severe anaphylactic reaction, associated with the simultaneous appearance of factor IX inhibitor (see Warnings and Precautions for Use section ). The required treatment depends on the nature and severity of the reaction.
It has been described following the induction of immune tolerance, the occurrence of a nephrotic syndrome in patients with hemophilia B exhibiting a factor IX inhibitor.
Rare cases of hyperthermia can be observed.
Patients with hemophilia B may develop anti-factor IX antibodies (inhibitors).
The appearance of such inhibitors results in an insufficient clinical response. In this case, it is recommended to contact a center specialized in the treatment of hemophilia.
In clinical studies in 11 previously untreated patients, no inhibitors were detected following the injection of BETAFACT.
Administration of factor IX includes a potential risk of thromboembolic complications, particularly with low purity preparations. The occurrence of myocardial infarction, disseminated intravascular coagulation, venous thrombosis and pulmonary embolism could be correlated with the use of low purity factor IX preparations. Administration of highly purified human coagulation factor IX, such as BETAFACT, is rarely associated with such adverse effects.
For safety regarding transmissible agents, see Warnings and precautions for use .