Von Willebrand factor

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Von Willebrand factor
Adult Indications & Dosage
Pediatric Indications & Dosage
Contraindications
Warnings & Precautions
Adverse Reactions
Drug Interactions
Use in Specific Populations
Administration & Monitoring
Overdosage
Pharmacology
Clinical Studies
How Supplied
Images
Patient Counseling Information
Precautions with Alcohol
Brand Names
Look-Alike Names

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Alberto Plate [2]

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Overview

Von Willebrand factor is a plasma derivate that is FDA approved for the treatment of hemophilia A and surgical and/or invasive procedures in adult and pediatric patients with von Willebrand Disease in whom desmopressin (DDAVP) is either ineffective or contraindicated. Common adverse reactions include facial edema, pruritus, rash, urticaria, nausea, dizziness, headache, paresthesia, pharyngitis, pain, shivering and factor VIII disorder.

Adult Indications and Dosage

FDA-Labeled Indications and Dosage (Adult)

Hemophilia A: Control and prevention of bleeding episodes
  • Dose (units) = body weight (kg) x desired FVIII rise (IU/dL or % of normal) x 0.5 (IU/kg per IU/dL).
    • Frequency of intravenous injection of the reconstituted product is determined by the type of bleeding episode and the recommendation of the treating physician.
Von Willebrand Disease: Surgical and/or Invasive Procedure in Adult and Pediatric patients
  • Dosage: Pre-operative dose of 60 IU VWF:RCo/kg body weight; subsequent doses of 40-60 IU VWF:RCo/kg body weight at 8-12 hour intervals post-operative as clinically needed.

Off-Label Use and Dosage (Adult)

Guideline-Supported Use

There is limited information regarding Off-Label Guideline-Supported Use of Von Willebrand factor in adult patients.

Non–Guideline-Supported Use

There is limited information regarding Off-Label Non–Guideline-Supported Use of Von Willebrand factor in adult patients.

Pediatric Indications and Dosage

FDA-Labeled Indications and Dosage (Pediatric)

Von Willebrand Disease: Surgical and/or Invasive Procedure in Adult and Pediatric patients
  • Dosage: Pre-operative dose of 75 IU VWF:RCo/kg body weight; subsequent doses of 50-75 IU VWF:RCo/kg body weight at 8-12 hour intervals post-operative as clinically needed.

Off-Label Use and Dosage (Pediatric)

Guideline-Supported Use

There is limited information regarding Off-Label Guideline-Supported Use of Von Willebrand factor in pediatric patients.

Non–Guideline-Supported Use

There is limited information regarding Off-Label Non–Guideline-Supported Use of Von Willebrand factor in pediatric patients.

Contraindications

  • Von Willebrand factor is contraindicated in patients who have manifested life-threatening immediate hypersensitivity reactions, including anaphylaxis, to the product or its components.

Warnings

Anaphylaxis and Severe Hypersensitivity Reactions
  • Anaphylaxis and severe hypersensitivity reactions are possible. Should symptoms occur, treatment with Von Willebrand factor should be discontinued, and emergency treatment should be administered.
Neutralizing Antibodies
  • Development of procoagulant activity-neutralizing antibodies (inhibitors) has been detected in patients receiving FVIII-containing products. Carefully monitor patients treated with AHF products for the development of FVIII inhibitors by appropriate clinical observations and laboratory tests. No studies have been conducted with Von Willebrand factor to evaluate inhibitor formation. Therefore, it is not known whether there are greater, lesser or the same risks of developing inhibitors due to the use of this product than there are with other FVIII preparations. If expected plasma FVIII activity levels are not attained, or if bleeding is not controlled with an appropriate dose, an assay that measures FVIII inhibitor concentration should be performed. Patients with these inhibitors may not respond to treatment with Antihemophilic Factor/von Willebrand Factor Complex (Human), or the response may be much less than would otherwise be expected; therefore, larger doses of Antihemophilic Factor/von Willebrand Factor Complex (Human) are often required. The management of bleeding in patients with inhibitors requires careful monitoring, especially if surgical procedures are indicated. Depending on the level of the inhibitor and/or clinical response, it may be appropriate to use an alternative ‘bypass’ therapeutic agent.
  • Reports in the literature suggest that patients with Type 3, severe von Willebrand Disease, may develop alloantibodies to von Willebrand factor (VWF) after replacement therapy. The risk of developing alloantibodies in patients with von Willebrand disease due to the use of this product is not known.
Thromboembolic Events
  • Thromboembolic events have been reported in von Willebrand Disease patients receiving AHF/VWF Complex (Human) replacement therapy, especially in the setting of known risk factors for thrombosis. In addition, endogenous high levels of FVIII have also been associated with thrombosis but no causal relationship has been established. In all VWD patients in situations of high thrombotic risk receiving coagulation factor replacement therapy, caution should be exercised and antithrombotic measures should be considered.
Intravascular Hemolysis
  • Massive doses of AHF/VWF Complex (Human) have resulted in a few cases of acute hemolytic anemia, increased bleeding tendency or hyperfibrinogenemia as reported in the literature, which subside after cessation of the commercial factor infusion. Von Willebrand factor contains blood group specific isoagglutinins and, when large and/or frequent doses are required in patients of blood groups A, B, or AB, the patient should be monitored for signs of intravascular hemolysis and falling hematocrit. Should this condition occur, thus leading to progressive hemolytic anemia, the administration of serologically compatible Type O red blood cells should be considered, the administration of Von Willebrand factor should be discontinued, and alternative therapy should be considered.
Vasomotor Reactions
  • Rapid administration of a FVIII concentrate may result in vasomotor reactions. Von Willebrand factor should not be administered at a rate exceeding 10 mL/minute.
Transmissible Infectious Agents
  • Because Von Willebrand factor is made from pooled human plasma, it may carry a risk of transmitting infectious agents, e.g., viruses, and theoretically, the Creutzfeldt-Jakob Disease (CJD) agent. Stringent procedures designed to reduce the risk of adventitious agent transmission have been employed in the manufacture of this product, from the screening of plasma donors and the collection and testing of plasma, through the application of viral elimination/reduction steps such as solvent detergent and heat treatment in the manufacturing process. Despite these measures, such products can still potentially transmit disease; therefore, the risk of infectious agents cannot be totally eliminated.

Adverse Reactions

Clinical Trials Experience

Respiratory System
Gastrointestinal Effects
Dermatological Effects
Neurological Effects
General Symptoms
Musculoeskeletal Effects
Hematological and Lymphatic Effects
Urogenital Effects
Special Senses

Postmarketing Experience

There is limited information regarding Von Willebrand factor Postmarketing Experience in the drug label.

Drug Interactions

There is limited information regarding Von Willebrand factor Drug Interactions in the drug label.

Use in Specific Populations

Pregnancy

Pregnancy Category (FDA): There is no FDA guidance on usage of Von Willebrand factor in women who are pregnant.
Pregnancy Category (AUS): There is no Australian Drug Evaluation Committee (ADEC) guidance on usage of Von Willebrand factor in women who are pregnant.

Labor and Delivery

There is no FDA guidance on use of Von Willebrand factor during labor and delivery.

Nursing Mothers

There is no FDA guidance on the use of Von Willebrand factor in women who are nursing.

Pediatric Use

There is no FDA guidance on the use of Von Willebrand factor in pediatric settings.

Geriatic Use

There is no FDA guidance on the use of Von Willebrand factor in geriatric settings.

Gender

There is no FDA guidance on the use of Von Willebrand factor with respect to specific gender populations.

Race

There is no FDA guidance on the use of Von Willebrand factor with respect to specific racial populations.

Renal Impairment

There is no FDA guidance on the use of Von Willebrand factor in patients with renal impairment.

Hepatic Impairment

There is no FDA guidance on the use of Von Willebrand factor in patients with hepatic impairment.

Females of Reproductive Potential and Males

There is no FDA guidance on the use of Von Willebrand factor in women of reproductive potentials and males.

Immunocompromised Patients

There is no FDA guidance one the use of Von Willebrand factor in patients who are immunocompromised.

Administration and Monitoring

Administration

There is limited information regarding Von Willebrand factor Administration in the drug label.

Monitoring

There is limited information regarding Von Willebrand factor Monitoring in the drug label.

IV Compatibility

There is limited information regarding the compatibility of Von Willebrand factor and IV administrations.

Overdosage

There is limited information regarding Von Willebrand factor overdosage. If you suspect drug poisoning or overdose, please contact the National Poison Help hotline (1-800-222-1222) immediately.

Pharmacology

There is limited information regarding Von Willebrand factor Pharmacology in the drug label.

Mechanism of Action

There is limited information regarding Von Willebrand factor Mechanism of Action in the drug label.

Structure

There is limited information regarding Von Willebrand factor Structure in the drug label.

Pharmacodynamics

There is limited information regarding Von Willebrand factor Pharmacodynamics in the drug label.

Pharmacokinetics

There is limited information regarding Von Willebrand factor Pharmacokinetics in the drug label.

Nonclinical Toxicology

There is limited information regarding Von Willebrand factor Nonclinical Toxicology in the drug label.

Clinical Studies

There is limited information regarding Von Willebrand factor Clinical Studies in the drug label.

How Supplied

There is limited information regarding Von Willebrand factor How Supplied in the drug label.

Storage

There is limited information regarding Von Willebrand factor Storage in the drug label.

Images

Drug Images

Package and Label Display Panel

Patient Counseling Information

There is limited information regarding Von Willebrand factor Patient Counseling Information in the drug label.

Precautions with Alcohol

Alcohol-Von Willebrand factor interaction has not been established. Talk to your doctor about the effects of taking alcohol with this medication.

Brand Names

There is limited information regarding Von Willebrand factor Brand Names in the drug label.

Look-Alike Drug Names

There is limited information regarding Von Willebrand factor Look-Alike Drug Names in the drug label.

Drug Shortage Status

Price

References

The contents of this FDA label are provided by the National Library of Medicine.


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