Cardiff-led research underpins new treatment and management of acquired haemophilia A
Defining the standard of care for acquired haemophilia A
Acquired haemophilia A (AHA) is an autoimmune disease caused by antibodies to clotting factor VIII. It is associated with severe bleeding and may be caused by underlying autoimmune disease, malignancy or pregnancy. Around 1.5 million mainly older people are affected, with a 30-40% mortality rate every year.
Before our research programme, the description of AHA was limited to single centre cohorts and collections of referral centre experiences, reflecting only the more severely affected patients. This information was not applicable to most patients as studies had a short follow-up and long-term outcomes were unknown.
There was only one controlled study in the field and this was underpowered and uninformative. Management guidelines therefore relied on expert opinion rather than evidence. To define standards of care for AHA based on evidence, our research team initiated a programme which evolved into UK and Europe-wide collaborations.
For the first time, our data and research led by Professor Peter Collins and colleague Angela Huth-Kuhne in Germany, allowed for evidence-based treatment of AHA. It was previously common when patients were tested for abnormal clotting agents to have their results delayed. Now our research guidelines advise laboratories to investigate abnormal clotting tests for AHA - even if the referring clinician had not requested them.
These guidelines have also been endorsed by the British Committee on Standards in Haematology, UKHCDO and the British Society of Haematology. They have also been agreed and adopted by the English Specialised Commissioning Group (The Haemophilia Clinical Reference Group) which has directed that all 90 UK haemophilia centres must follow UKHCDO guidelines.
Our research programme has also resulted in the production of two international collaborations between Cardiff University and European, north American and Japanese colleagues.
Treatment guidelines disseminated globally to patient groups, clinicians and physicians.
Our global impact
Our research guidelines are the most cited papers in the field during the last five years. They have been disseminated to patient groups and adopted by clinicians specialising in haemostasis and general physicians around the world and cited by groups from Europe,and south America, India, Korea and Japan as accepted standard practice.
- Tengborn, L. et al., 2012. Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry. BJOG: An International Journal of Obstetrics & Gynaecology 119 (12), pp.1529-1537. (10.1111/j.1471-0528.2012.03469.x)
- Baudo, F. et al., 2012. Management of bleeding in acquired hemophilia A: Results from the European Acquired Haemophilia (EACH2) Registry. Blood 120 (1), pp.39-46. (10.1182/blood-2012-02-408930)
- Collins, P. W. et al. 2012. Immunosuppression for acquired hemophilia A: Results from the European Acquired Haemophilia Registry (EACH2). Blood 120 (1), pp.47-55. (10.1182/blood-2012-02-409185)
- Knoebl, P. et al., 2012. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). Journal of Thrombosis and Haemostatis 10 (4), pp.622-631. (10.1111/j.1538-7836.2012.04654.x)
- Collins, P. W. et al. 2004. A population based, unselected, consecutive cohort of patients with acquired haemophilia A. British Journal of Haematology 124 (1), pp.86-90. (10.1046/j.1365-2141.2003.04731.x)