Abstract

Mild haemophilia is defined by factor levels between 0.05 and 0.40 IU/mL and is characterised by traumatic bleeds. Major issues associated with mild haemophilia are that it may not present for many years after birth, and that awareness, even within families, may be low. Methodological problems exist in diagnosis, such as inconsistencies in results obtained from different assays used to measure factor levels in mild haemophilia. Advances in genetic testing provide insight into diagnosis as well as the likelihood of inhibitor development, which is not uncommon in patients with mild or moderate haemophilia and can increase morbidity. The management of patients with mild haemophilia is a challenge. This review includes suggestions around formulating treatment plans for these patients, encompassing the full spectrum from clinical care of the newly diagnosed neonate to that of the ageing patient with multiple comorbidities. Management strategies consider not only the vast differences in these patients' needs, but also risks of inhibitor development and approaches to optimally engage patients.

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Authors

Gary Benson - Haemophilia and Thrombosis Centre, Belfast City Hospital, Belfast, Northern Ireland, United Kingdom

Günter x Günter Auerswald - Klinikum Bremen-Mitte, Professor Hess Children's Hospital, Bremen, Germany

Gerry Dolan - Centre for Haemostasis and Thrombosis, St Thomas' Hospital, London, United Kingdom

Anne Duffy - WFH Psychosocial Committee, Irish Haemophilia Society, Dublin, Ireland

Cedric Hermans - Haemostasis and Thrombosis Unit, Division of Haematology, Cliniques Universitaires Saint-Luc, Brussels, Belgium

Rolf Ljung - Department of Paediatrics, Lund University, Lund, Sweden; Malmö Centre for Thrombosis and Haemostasis, Skåne University Hospital, Malmö, Sweden

Massimo Morfini - Italian Association of Haemophilia Centres, Florence, Italy

Silva Zupančić Šalek - Unit for Haemostasis, Thrombosis and Benign Diseases of Haematopoietic System, Division of Haematology, Department of Internal Medicine, University Hospital Centre Zagreb, Zagreb, Croatia; Medical School University of Zagreb, Zagreb, Croatia; Faculty of Medicine Osijek, J.J. Strossmayer University of Osijek, Osijek, Croatia

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