Blog

December 10, 2018
ISTH rethinks definition and diagnosis of mild haemophilia A

The internationally recognised definition of mild haemophilia A – a FVIII level between of 5 and 40 IU/dL – takes no account of the assay used to measure FVIII. As a result, according to the International Society for Thrombosis and Haemostasis (ISTH), FVIII measurements can be imprecise due to variation in gene mutations (J Thromb …

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Long term study shows improvements in outcome in Denmark

The progress of people with moderate to severe haemophilia in Denmark has been followed in surveys conducted in 1988 (n=128), 2001 (n=156, 94% severe) and 2012 (n=164, 74% severe) (Acta Haematol 2018;140:240-246). The findings show that proportion of the patient population falling within the oldest age group (55 – 88 years) increased from 4% of …

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WFH publishes 2017 global survey

The World Federation for Hemophilia has published its latest survey – the 19th – of worldwide demographics and treatment data for people with bleeding disorders. The survey, available in print and online at www.wfh.org/globalsurvey, includes data on haemophilia, von Willebrand disease and rare factor deficiencies and inherited platelet disorders affecting 315,000 people in 116 countries. This …

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November 6, 2018
Adherence and factor replacement therapy

About one-fifth of adults, young people and children have problems with adherence to factor replacement therapy, according to a systematic review of 16 studies from Canada (Patient Prefer Adherence 2018 Oct 8; doi: 10.2147/PPA.S177624). The analysis, using a framework adapted from research in people with diabetes, found that some studies had focused attention on factors …

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Exercise and haemophilia

The haemophilia community is now past the stage where opinion leaders say exercise is a good thing, with increased activity and sports participation widely promoted for everyone – as appropriate individually, of course. A recent review from Sweden provides a marker of progress in research and clinical thinking to date (Semin Thromb Hemost 2018 Oct …

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rVWF prophylaxis during elective surgery

A multinational trial involving eleven centres, including NHS services in Oxford and London, has shown that recombinant von Willebrand factor (rVWF) is safe and effective as prophylaxis during elective surgery in people with severe von Willebrand’s disease. Fifteen patients undergoing major, minor or oral surgery received rVWF 40-60 IU/kg one hour before surgery and postoperatively …

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Update on trials of EHL Factor IX

The extended half-life (EHL) factors are being introduced with a sometimes bewildering amount of supporting information and advice, not least the ever-growing evidence base of clinical trials. A new review provides a convenient summary of three ongoing trial programmes – BLONG (eftrenonacog alfa, Alprolix), PROLONG-9FP (albutrepenonacog alfa, Idelvion) and PARADIGM (nonacog beta pegol, Refixia) (Ther …

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Management of the foetus and newborn with haemophilia

Managing haemophilia during pregnancy and in a newborn child involves a wide range of health disciplines and this, say Canadian clinicians, plus a lack of high quality information needed to inform decision-making, creates a correspondingly wide variation in clinical practice (Clin Appl Thromb Hemost 2018 Oct 29: doi: 10.1177/1076029618807583). Their recommendations cover everything from preconception …

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Intracranial haemorrhage in children with haemophilia A

A study from China has described the epidemiology and risk factors for intracranial haemorrhage (ICH) in children with haemophilia A (Curr Med Sci 2018;38:875-879). The retrospective study identified 126 children with haemophilia A in one centre in the past four years, of whom 36 received factor VIII prophylaxis. None of those children had ICH but …

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Bleeding risk in patients with haemophilia taking antithrombotic therapy

Drug therapy to reduce the risk of thrombosis, such as antiplatelet therapy with aspirin or anticoagulant therapy, is an important component of preventing cardiovascular events in people at increased risk. There is a lack of information about how such treatment might affect the risk of bleeding in people with haemophilia, who can now expect to …

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