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Diagnosis and care of patients with mild haemophilia: practical recommendations for clinical management.
Benson, Gary; Auerswald, Günter; Dolan, Gerry; Duffy, Anne; Hermans, Cedric; Ljung, Rolf; Morfini, Massimo; Salek, Silva Zupancic.
Afiliación
  • Benson G; Haemophilia and Thrombosis Centre, Belfast City Hospital, Belfast, Northern Ireland, United Kingdom.
  • Auerswald G; Klinikum Bremen-Mitte, Professor Hess Children's Hospital, Bremen, Germany.
  • Dolan G; Centre for Haemostasis and Thrombosis, St Thomas' Hospital, London, United Kingdom.
  • Duffy A; WFH Psychosocial Committee, Irish Haemophilia Society, Dublin, Ireland.
  • Hermans C; Haemostasis and Thrombosis Unit, Division of Haematology, Cliniques Universitaires Saint-Luc, Brussels, Belgium.
  • Ljung R; Department of Paediatrics, Lund University, Lund, Sweden.
  • Morfini M; Malmö Centre for Thrombosis and Haemostasis, Skåne University Hospital, Malmö, Sweden.
  • Salek SZ; Italian Association of Haemophilia Centres, Florence, Italy.
Blood Transfus ; 16(6): 535-544, 2018 11.
Article en En | MEDLINE | ID: mdl-29328905
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.
Asunto(s)

Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Factores de Coagulación Sanguínea / Hemofilia A Tipo de estudio: Diagnostic_studies / Guideline Límite: Humans Idioma: En Revista: Blood Transfus Año: 2018 Tipo del documento: Article País de afiliación: Reino Unido

Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Factores de Coagulación Sanguínea / Hemofilia A Tipo de estudio: Diagnostic_studies / Guideline Límite: Humans Idioma: En Revista: Blood Transfus Año: 2018 Tipo del documento: Article País de afiliación: Reino Unido