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)
- author
- Benson, Gary
; Auerswald, Günter
; Dolan, Gerry
; Duffy, Anne
; Hermans, Cedric
; Ljung, Rolf
LU
; Morfini, Massimo
and Šalek, Silva Zupančić
- organization
- publishing date
- 2018
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Blood Transfusion
- pages
- 535 - 544
- publisher
- Simiti Servizi SRL
- external identifiers
-
- pmid:29328905
- 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
- 2024-06-10 19:54:59
@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}},
doi = {{10.2450/2017.0150-17}},
year = {{2018}},
}