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How I manage patients with inherited haemophilia A and B and factor inhibitors

Ljung, Rolf C.R. LU (2018) In British Journal of Haematology 180(4). p.501-510
Abstract

Development of inhibitors to coagulation factor VIII or IX is still the most challenging complication in haemophilia care. 'Bypassing agents' may be used to treat a bleed but the eradication of the inhibitor by immune tolerance induction (ITI) is the main objective in the treatment of a patient with haemophilia who has developed neutralizing antibodies. Several options exist for ITI and the patient may be at 'good' or 'bad risk' for successful outcome with different regimens. This paper offers a review of current regimens to be considered in the treatment of a bleed in a patient with an inhibitor but the main focus is the aspects of different choices in the management of the child or the adult with severe or mild forms of haemophilia A... (More)

Development of inhibitors to coagulation factor VIII or IX is still the most challenging complication in haemophilia care. 'Bypassing agents' may be used to treat a bleed but the eradication of the inhibitor by immune tolerance induction (ITI) is the main objective in the treatment of a patient with haemophilia who has developed neutralizing antibodies. Several options exist for ITI and the patient may be at 'good' or 'bad risk' for successful outcome with different regimens. This paper offers a review of current regimens to be considered in the treatment of a bleed in a patient with an inhibitor but the main focus is the aspects of different choices in the management of the child or the adult with severe or mild forms of haemophilia A or B, who has developed an inhibitor. There are also some final outlooks on new and emerging treatment possibilities.

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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Factor IX, Factor VIII, Haemophilia, Immune tolerance induction, Inhibitors
in
British Journal of Haematology
volume
180
issue
4
pages
501 - 510
publisher
Federation of European Neuroscience Societies and Blackwell Publishing Ltd
external identifiers
  • scopus:85038840406
ISSN
0007-1048
DOI
10.1111/bjh.15053
language
English
LU publication?
yes
id
ab634bd5-7099-4d0a-bb88-b38b1d695628
date added to LUP
2018-01-10 16:21:06
date last changed
2018-02-09 10:52:27
@article{ab634bd5-7099-4d0a-bb88-b38b1d695628,
  abstract     = {<p>Development of inhibitors to coagulation factor VIII or IX is still the most challenging complication in haemophilia care. 'Bypassing agents' may be used to treat a bleed but the eradication of the inhibitor by immune tolerance induction (ITI) is the main objective in the treatment of a patient with haemophilia who has developed neutralizing antibodies. Several options exist for ITI and the patient may be at 'good' or 'bad risk' for successful outcome with different regimens. This paper offers a review of current regimens to be considered in the treatment of a bleed in a patient with an inhibitor but the main focus is the aspects of different choices in the management of the child or the adult with severe or mild forms of haemophilia A or B, who has developed an inhibitor. There are also some final outlooks on new and emerging treatment possibilities.</p>},
  author       = {Ljung, Rolf C.R.},
  issn         = {0007-1048},
  keyword      = {Factor IX,Factor VIII,Haemophilia,Immune tolerance induction,Inhibitors},
  language     = {eng},
  number       = {4},
  pages        = {501--510},
  publisher    = {Federation of European Neuroscience Societies and Blackwell Publishing Ltd},
  series       = {British Journal of Haematology},
  title        = {How I manage patients with inherited haemophilia A and B and factor inhibitors},
  url          = {http://dx.doi.org/10.1111/bjh.15053},
  volume       = {180},
  year         = {2018},
}