THE BENEFITS OF FACTOR VIII PROPHYLAXIS: THE LATEST WFH RECOMMENDATIONS

Prophylaxis has been conventionally defined as the regular intravenous infusion of missing coagulation Factor VIII (FVIII) in persons with haemophilia A and Factor IX (FIX) in persons with haemophilia B, administered to increase their levels, with the intent of preventing bleeding.

For patients with haemophilia A or B with a severe phenotype, the World Federation of Hemophilia (WFH) strongly recommends prophylaxis sufficient to prevent bleeding at all times that is individualised and takes into account the patient’s bleeding, joint status, individual pharmacokinetics, and personal needs.

For paediatric patients with severe haemophilia type A or B, the WFH recommends that prophylaxis be initiated early with coagulation factor concentrates, or other haemostatic agents, before the onset of joint disease and preferably before age 3 years, in order to prevent haemarthrosis that may lead to joint degeneration.

For people with severe haemophilia on prophylaxis, WFH recommends that both patients and caregivers be taught to promptly and accurately record all bleeding episodes and treatments, and further recommends that patients be followed in specialised haemophilia treatment centres.

For patients who, despite good adherence to prophylaxis, still experience bleeding, WFH recommends intensifying prophylaxis and, if necessary, orthopaedic intervention.

 

Reference:

  • WFH Guidelines for the Management of Hemophilia, 3rd edition, Chapter 6: Prophylaxis in Hemophilia, Carcao et. al, Haemophilia. 2020;26(Suppl 6):1–158., DOI: 10.1111/hae.14046
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Wikiphilia

Learn more about the meaning of the words you read on this page and learn about the entire glossary on hemophilia.

A protein belonging to the clotting factor category, a group of enzymes involved in the blood-clotting process.

It is encoded by a gene on the long arm of the X chromosome.

Factor VIII is also known as anti-haemophilic factor (AHF).

Product containing a high concentration of freeze-dried factor VIII, used for replacement therapy in the treatment of haemophilia A.

A ereditary genetic illness, characterised by a deficiency of clotting factor VIII, that exposes the individual to a greater risk of both internal and external bleeds.

Haemophilia A is more common in males, whereas females tend to be healthy carriers of the condition.

The typical symptoms of the condition include haemarthroses (joint bleeds) and haematomas (muscle bleeds).

Condition that occurs when factor VIII activity is between 1% and 5%.

Condition that occurs when factor VIII activity is between 1% and 5%.

Prophylactic treatment containing replacement factor VIII that is commenced after the onset of the first haemarthrosis, before the appearance of documented joint disease, and in any case before the age of 3 years.

Treatment regimen usually used in patients with severe haemophilia A.

It involves the constant, regular administration of the lacking factor VIII, in order to prevent bleeds.

Prophylactic treatment containing replacement factor VIII that is commenced after the onset of at least two haemarthroses, but before the appearance of documented joint disease.

Trattamento profilattico a base di fattore VIII sostitutivo iniziato in seguito alla comparsa di patologie articolari documentate.