Advanced

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 LU ; Morfini, Massimo and Šalek, Silva Zupančić (2018) In Blood Transfusion p.535-544
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... (More)

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.

(Less)
Please use this url to cite or link to this publication:
author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Blood Transfusion
pages
535 - 544
publisher
SIMITI SERVIZI SRL
external identifiers
  • scopus:85056036898
ISSN
1723-2007
DOI
10.2450/2017.0150-17
language
English
LU publication?
yes
id
6c2965fc-c278-4861-bc04-31228ea1a826
date added to LUP
2018-10-12 12:13:33
date last changed
2019-04-23 04:40:25
@article{6c2965fc-c278-4861-bc04-31228ea1a826,
  abstract     = {<p>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.</p>},
  author       = {Benson, Gary and Auerswald, Günter and Dolan, Gerry and Duffy, Anne and Hermans, Cedric and Ljung, Rolf and Morfini, Massimo and Šalek, Silva Zupančić},
  issn         = {1723-2007},
  language     = {eng},
  pages        = {535--544},
  publisher    = {SIMITI SERVIZI SRL},
  series       = {Blood Transfusion},
  title        = {Diagnosis and care of patients with mild haemophilia : practical recommendations for clinical management},
  url          = {http://dx.doi.org/10.2450/2017.0150-17},
  year         = {2018},
}