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Pathogenesis and Therapeutic Mechanisms in  Immune Thrombocytopenia (ITP)

Zufferey, Anne ; Kapur, Rick LU and Semple, John W LU (2017) In Journal of Clinical Medicine 6(2).
Abstract

Immune thrombocytopenia (ITP) is a complex autoimmune disease characterized by low platelet counts. The pathogenesis of ITP remains unclear although both antibody-mediated and/or T cell-mediated platelet destruction are key processes. In addition, impairment of T cells, cytokine imbalances, and the contribution of the bone marrow niche have now been recognized to be important. Treatment strategies are aimed at the restoration of platelet counts compatible with adequate hemostasis rather than achieving physiological platelet counts. The first line treatments focus on the inhibition of autoantibody production and platelet degradation, whereas second-line treatments include immunosuppressive drugs, such as Rituximab, and splenectomy.... (More)

Immune thrombocytopenia (ITP) is a complex autoimmune disease characterized by low platelet counts. The pathogenesis of ITP remains unclear although both antibody-mediated and/or T cell-mediated platelet destruction are key processes. In addition, impairment of T cells, cytokine imbalances, and the contribution of the bone marrow niche have now been recognized to be important. Treatment strategies are aimed at the restoration of platelet counts compatible with adequate hemostasis rather than achieving physiological platelet counts. The first line treatments focus on the inhibition of autoantibody production and platelet degradation, whereas second-line treatments include immunosuppressive drugs, such as Rituximab, and splenectomy. Finally, thirdline treatments aim to stimulate platelet production by megakaryocytes. This review discusses the pathophysiology of ITP and how the different treatment modalities affect the pathogenic mechanisms.

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author
; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
immune thrombocytopenia (ITP), autoimmunity, T cells, B cells, platelets
in
Journal of Clinical Medicine
volume
6
issue
2
pages
21 pages
publisher
MDPI AG
external identifiers
  • scopus:85067220122
  • pmid:28208757
ISSN
2077-0383
DOI
10.3390/jcm6020016
language
English
LU publication?
yes
id
f2e314b1-881e-4bb1-8640-59beb37ad4d0
date added to LUP
2022-11-09 15:21:29
date last changed
2024-04-19 21:00:42
@article{f2e314b1-881e-4bb1-8640-59beb37ad4d0,
  abstract     = {{<p>Immune thrombocytopenia (ITP) is a complex autoimmune disease characterized by low platelet counts. The pathogenesis of ITP remains unclear although both antibody-mediated and/or T cell-mediated platelet destruction are key processes. In addition, impairment of T cells, cytokine imbalances, and the contribution of the bone marrow niche have now been recognized to be important. Treatment strategies are aimed at the restoration of platelet counts compatible with adequate hemostasis rather than achieving physiological platelet counts. The first line treatments focus on the inhibition of autoantibody production and platelet degradation, whereas second-line treatments include immunosuppressive drugs, such as Rituximab, and splenectomy. Finally, thirdline treatments aim to stimulate platelet production by megakaryocytes. This review discusses the pathophysiology of ITP and how the different treatment modalities affect the pathogenic mechanisms.</p>}},
  author       = {{Zufferey, Anne and Kapur, Rick and Semple, John W}},
  issn         = {{2077-0383}},
  keywords     = {{immune thrombocytopenia (ITP); autoimmunity; T cells; B cells; platelets}},
  language     = {{eng}},
  month        = {{02}},
  number       = {{2}},
  publisher    = {{MDPI AG}},
  series       = {{Journal of Clinical Medicine}},
  title        = {{Pathogenesis and Therapeutic Mechanisms in  Immune Thrombocytopenia (ITP)}},
  url          = {{http://dx.doi.org/10.3390/jcm6020016}},
  doi          = {{10.3390/jcm6020016}},
  volume       = {{6}},
  year         = {{2017}},
}