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Recent advances in the mechanisms and treatment of immune thrombocytopenia

Provan, Drew and Semple, John W LU (2022) In EBioMedicine 76. p.1-10
Abstract

Primary immune thrombocytopenia is an autoimmune disease associated with a reduced peripheral blood platelet count. The phenotype is variable with some patients suffering no bleeding whilst others have severe bleeding which may be fatal. Variability in clinical behaviour and treatment responses reflects its complex underlying pathophysiology. Historically the management has relied heavily on immune suppression. Recent studies have shown that the older empirical immune suppressants fail to alter the natural history of the disease and are associated with a poor quality of life for patients. Newer treatments, such as the thrombopoietin receptor agonists, have transformed ITP care. They have high efficacy, are well tolerated and improve... (More)

Primary immune thrombocytopenia is an autoimmune disease associated with a reduced peripheral blood platelet count. The phenotype is variable with some patients suffering no bleeding whilst others have severe bleeding which may be fatal. Variability in clinical behaviour and treatment responses reflects its complex underlying pathophysiology. Historically the management has relied heavily on immune suppression. Recent studies have shown that the older empirical immune suppressants fail to alter the natural history of the disease and are associated with a poor quality of life for patients. Newer treatments, such as the thrombopoietin receptor agonists, have transformed ITP care. They have high efficacy, are well tolerated and improve patients' quality of life. A greater understanding of the underlying pathophysiology of this disorder has helped develop a number of new targeted therapies. These include inhibitors of the neonatal Fc receptor inhibitors, Bruton tyrosine kinase and complement pathway. Here we discuss the mechanisms underlying ITP and the new approach to ITP care.

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author
and
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
EBioMedicine
volume
76
article number
103820
pages
1 - 10
publisher
Elsevier
external identifiers
  • scopus:85123080690
  • pmid:35074629
ISSN
2352-3964
DOI
10.1016/j.ebiom.2022.103820
language
English
LU publication?
yes
additional info
Copyright © 2022 The Author(s). Published by Elsevier B.V. All rights reserved.
id
5ef52376-aee8-4344-a126-cc394b4c5249
date added to LUP
2022-03-18 13:14:00
date last changed
2024-06-16 23:19:32
@article{5ef52376-aee8-4344-a126-cc394b4c5249,
  abstract     = {{<p>Primary immune thrombocytopenia is an autoimmune disease associated with a reduced peripheral blood platelet count. The phenotype is variable with some patients suffering no bleeding whilst others have severe bleeding which may be fatal. Variability in clinical behaviour and treatment responses reflects its complex underlying pathophysiology. Historically the management has relied heavily on immune suppression. Recent studies have shown that the older empirical immune suppressants fail to alter the natural history of the disease and are associated with a poor quality of life for patients. Newer treatments, such as the thrombopoietin receptor agonists, have transformed ITP care. They have high efficacy, are well tolerated and improve patients' quality of life. A greater understanding of the underlying pathophysiology of this disorder has helped develop a number of new targeted therapies. These include inhibitors of the neonatal Fc receptor inhibitors, Bruton tyrosine kinase and complement pathway. Here we discuss the mechanisms underlying ITP and the new approach to ITP care.</p>}},
  author       = {{Provan, Drew and Semple, John W}},
  issn         = {{2352-3964}},
  language     = {{eng}},
  pages        = {{1--10}},
  publisher    = {{Elsevier}},
  series       = {{EBioMedicine}},
  title        = {{Recent advances in the mechanisms and treatment of immune thrombocytopenia}},
  url          = {{http://dx.doi.org/10.1016/j.ebiom.2022.103820}},
  doi          = {{10.1016/j.ebiom.2022.103820}},
  volume       = {{76}},
  year         = {{2022}},
}