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VONCENTO is a brand name for Von Willebrand Factor. The medicine, its uses, side effects and dosage are the same regardless of brand.
Used for: Voncento can be used for all age groups. von Willebrand disease (VWD) Prophylaxis and treatment of haemorrhage or surgical bleeding in patients with VWD, when desmopressin (DDAVP) treatment alone is ineffective or contraindicated. Haemophilia A (congenital FVIII deficiency) Prophylaxis and treatment of bleeding in…
Verbatim from this product's MHRA label. Tap a section to expand.
Treatment of VWD and haemophilia A should be supervised by a physician experienced in the treatment of haemostatic disorders. The decision on the use of the product at home for patients with VWD and with haemophilia A should be made by the treating physician who should ensure that appropriate training is provided and the use is reviewed at intervals.
4. Treatment monitoring During the course of treatment, appropriate determination of factor VIII levels is advised to guide the dose to be administered and the frequency of repeated infusions. Individual patients may vary in their response to factor VIII, demonstrating different half-lives and recoveries.
Dose based on bodyweight may require adjustment in underweight or overweight patients. In the case of major surgical interventions in particular, precise monitoring of the substitution therapy by means of coagulation analysis (plasma factor VIII activity) is indispensable.
Posology von Willebrand disease It is important to calculate the dose using the number of IU of VWF:RCo specified. 02 IU/ml (2 %). 4 IU/ml (40 %) should be achieved. On-demand treatment Usually 40 - 80 IU/kg of von Willebrand factor (VWF:RCo) corresponding to 20 - 40 IU FVIII:C/kg of body weight (BW) are recommended to achieve haemostasis.
An initial dose of 80 IU/kg VWF:RCo may be required, especially in patients with type 3 VWD where maintenance of adequate levels may require greater doses than in other types of VWD. Prevention of haemorrhage in case of surgery For prevention of excessive bleeding during or after surgery the application should start 1 - 2 hours before the surgical procedure.
An appropriate dose should be re-administered every 12 - 24 hours. The dose and duration of the treatment depend on the clinical status of the patient, the type and severity of the bleeding, and both VWF:RCo and FVIII:C levels. When using a FVIII-containing VWF product, the treating physician should be aware that continued treatment may cause an excessive rise in FVIII:C.
2). Prophylaxis treatment For long term prophylaxis in patients with VWD, a dose of 25 - 40 IU VWF:RCo /kg body weight should be considered at a frequency of 1 to 3 times per week. In patients with gastrointestinal bleeds or menorrhagia, shorter dose intervals or higher doses may be necessary.
Summary of the safety profile During treatment with Voncento the following adverse reactions may occur: Hypersensitivity or allergic reactions, thromboembolic events, pyrexia, headache, dysgeusia and abnormal liver function test levels.
Furthermore patients may develop inhibitors to FVIII and VWF. Tabulated list of adverse reactions The table presented below is according to the MedDRA system organ classification. Frequencies have been evaluated according to the following convention: very common (≥1/10); common (≥1/100 to <1/10); uncommon (≥1/1,000 to <1/100); rare (≥1/10,000 to <1/1,000); very rare (<1/10,000), not known (cannot be estimated from the available data).
Within each frequency grouping, adverse reactions are presented in order of decreasing seriousness. MedDRA Standard System Organ Class Adverse Reaction* Frequency Blood and lymphatic system disorders FVIII inhibition VWF inhibition Uncommon (PTPs)** Very common (PUPs)** Not known*** Immune system disorders Hypersensitivity (including tachycardia, chest pain, chest discomfort and back pain) Common Nervous system disorders Dysgeusia Uncommon Vascular disorders Thromboembolic event Uncommon General disorders and administration site conditions Pyrexia Headache Common Very common Investigations Liver function test abnormal Uncommon *Adverse events assessed as related to administration of the Voncento **Frequency is based on studies with all FVIII products which included patients with severe haemophilia A.
PTPs = previously-treated patients, PUPs = previously- untreated patients. *** Observed during post-marketing surveillance, not observed in clinical trials. Description of selected adverse reactions Hypersensitivity (allergic 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 (including chest pain and chest discomfort), back pain, tingling, vomiting, wheezing) have been observed, and may in some cases progress to severe anaphylaxis (including shock).
Traceability It is strongly recommended that every time that Voncento is administered to a patient, the name and batch number of the product are recorded in order to maintain a link between the patient and the batch of the product.
Hypersensitivity Allergic type hypersensitivity reactions are possible. If symptoms of hypersensitivity occur, patients should be advised to discontinue use of the medicinal product immediately and contact their physician. Patients should be informed of the early signs of hypersensitivity reactions including hives, generalised urticaria, tightness of the chest, wheezing, hypotension and anaphylaxis.
In case of shock, the current medical standards for shock treatment should be observed. Virus safety Standard measures to prevent infections resulting from the use of medicinal products prepared from human blood or plasma include selection of donors, screening of individual donations and plasma pools for specific markers of infection and the inclusion of effective manufacturing steps for the inactivation/removal of viruses.
Despite this, when medicinal products prepared from human blood or plasma are administered, the possibility of transmitting infective agents cannot be totally excluded. This also applies to unknown or emerging viruses and other pathogens.
The measures taken are considered effective for enveloped viruses such as human immunodeficiency virus (HIV), hepatitis B virus (HBV) and hepatitis C virus (HCV), and for the non-enveloped hepatitis A virus (HAV). The measures taken may be of limited value against non-enveloped viruses such as parvovirus B19.
g. haemolytic anaemia). Appropriate vaccination (hepatitis A and B) should be considered for patients in regular/repeated receipt of human plasma-derived factor VIII/VWF products. von Willebrand disease There is a risk of occurrence of thrombotic events, particularly in patients with known clinical or laboratory risk factors.
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Not medical advice. Always read the patient information leaflet and follow your prescriber or pharmacist.
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The dose and duration of treatment will depend on the clinical status of the patient, as well as their VWF:RCo and FVIII:C plasma levels. Paediatric VWD population Treatment of bleeding Usually 40 - 80 IU/kg of von Willebrand factor (VWF:RCo) corresponding to 20 - 40 IU FVIII:C/kg of body weight (BW) are recommended in paediatric patients to treat a bleed.
Prophylaxis treatment Patients aged 12 to 18 years old:
Dosing is based on the same guidelines as for adults.
Patients aged <12 years old:
Based on results from a clinical trial in which paediatric patients under 12 years of age were shown to have lower exposure of VWF, a prophylactic dose range of 40 – 80 IU VWF:RCo/kg body weight 1 to 3 times a week should be considered.
2). The dose and duration of treatment will depend on the clinical status of the patient, as well as their VWF:RCo and FVIII:C plasma levels. Haemophilia A It is important to calculate the dose using the number of IU of FVIII:C specified.
The dose and duration of the substitution therapy depend on the severity of the factor VIII deficiency, on the location and extent of the bleeding and on the patient’s clinical condition. The number of units of factor VIII administered is expressed in International Units (IU), which is related to the current WHO concentrate standard for factor VIII products.
Factor VIII activity in plasma is expressed either as a percentage (relative to normal human plasma) or preferably in International Units (relative to an International Standard for factor VIII in plasma). 1 IU of factor VIII activity is equivalent to that quantity of factor VIII in 1 ml of normal human plasma.
On demand treatment The calculation of the required dose of factor VIII is based on the empirical finding that 1 International Unit (IU) factor VIII per kg body weight raises the plasma factor VIII activity by about 2 % of normal activity (in vivo recovery 2 IU/dl).
5. The amount to be administered and the frequency of administration should always be oriented to the clinical effectiveness in the individual case. In the case of the following haemorrhagic events, the factor VIII activity should not fall below the given plasma activity level (in % of normal or IU/dl) within the corresponding period.
The following table can be used to guide dosing in bleeding episodes and surgery:
Degree of haemorrhage / Type of surgical procedure Factor VIII level required (% or IU/dl) Frequency of doses (hours) / Duration of therapy (days) Haemorrhage Early haemarthrosis, muscle bleeding or oral bleeding 20 - 40 Repeat infusion every 12 - 24 hours for at least 1 day, until the bleeding episode as indicated by pain is resolved or healing is achieved.
More extensive haemarthrosis, muscle bleeding or haematoma 30 - 60 Repeat infusion every 12 - 24 hours for 3 - 4 days or more until pain and acute disability are resolved. Life-threatening haemorrhages 60 - 100 Repeat infusion every 8 - 24 hours until threat is resolved.
Surgery Minor surgery including tooth extraction 30 - 60 Repeat infusion every 24 hours for at least 1 day, until healing is achieved. […]
FVIII inhibition Development of neutralising antibodies (inhibitors) may occur in patients with haemophilia A treated with factor VIII, including with Voncento. If such inhibitors occur, the condition may manifest itself as an insufficient clinical response.
In such cases, it is recommended that a specialised haemophilia centre be contacted. VWF inhibition Patients with VWD, especially type 3 patients, may develop neutralising antibodies (inhibitors) to VWF. If such inhibitors occur, the condition will manifest itself as an inadequate clinical response.
Such antibodies are precipitating and may occur concomitantly to anaphylactic reactions. Therefore, patients experiencing an anaphylactic reaction should be evaluated for the presence of an inhibitor. In all such cases, it is recommended that a specialised haemophilia centre be contacted.
Thromboembolic events In patients with VWD, there is a risk of occurrence of thromboembolic events, particularly in patients with known clinical or laboratory risk factors. 4). 4. Paediatric population Frequency, type and severity of adverse reactions in children are expected to be the same as in adults.
Reporting of suspected adverse reactions Reporting suspected adverse reactions after authorisation of the medicinal product is important. It allows continued monitoring of the benefit/risk balance of the medicinal product. uk/yellowcard or search for MHRA Yellow Card in the Google Play or Apple App Store.
Therefore, patients at risk must be monitored for early signs of thrombosis. Prophylaxis against venous thromboembolism should be instituted, according to the current recommendations. When using a FVIII-containing VWF product, the treating physician should be aware that continued treatment may cause an excessive rise in FVIII:C.
2). Patients with VWD, especially type 3 patients, may develop neutralising antibodies (inhibitors) to VWF. If the expected VWF:RCo activity plasma levels are not attained, or if bleeding is not controlled with an appropriate dose, an appropriate assay should be performed to determine if a VWF inhibitor is present.
In patients with high levels of inhibitor, therapy may not only be ineffective but also lead to anaphylactoid reactions and other therapeutic options should be considered. Haemophilia A Inhibitors The formation of neutralising antibodies (inhibitors) to factor VIII is a known complication in the management of individuals with haemophilia A.
These inhibitors are usually IgG immunoglobulins directed against the factor VIII procoagulant activity, which are quantified in Bethesda Units (BU) per ml of plasma, using the modified assay. The risk of developing inhibitors is correlated to the severity of the disease as well as exposure to factor VIII, this risk being highest within the first 50 exposure days but continues throughout life although the risk is uncommon.
The clinical relevance of inhibitor development will depend on the titre of the inhibitor, with low titre posing less of a risk of insufficient clinical response than high titre inhibitors. In general, all patients treated with coagulation factor VIII products should be carefully monitored for the development of inhibitors by appropriate clinical observations and laboratory tests.
If the expected factor VIII activity plasma levels are not attained, or if bleeding is not controlled with an appropriate dose, testing for FVIII inhibitor presence should be performed. In patients with high levels of inhibitor, factor VIII therapy may not be effective and other therapeutic options should be considered.
Management of such patients should be directed by physicians with experience in the care of haemophilia and factor VIII inhibitors. Cardiovascular events In patients with existing cardiovascular risk factors, substitution therapy with FVIII may increase the cardiovascular risk.
Catheter-related complications If a central venous access device (CVAD) is required, risk of CVAD-related complications including local infections, bacteremia and catheter site thrombosis should be considered. 74% of the WHO recommended maximum daily intake of 2 g sodium for an adult.
48% of the WHO recommended maximum daily intake of 2 g sodium for an adult. Paediatric population The listed warnings and precautions apply both to adults and paediatrics.