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Autoimmune effector mechanisms associated with a defective immunosuppressive axis in immune thrombocytopenia (ITP)

Li, Qizhao ; Marcoux, Geneviève LU ; Hu, Yuefen ; Rebetz, Johan LU orcid ; Guo, Li LU ; Semple, Elisabeth ; Provan, Drew ; Xu, Shuqian ; Hou, Ming and Peng, Jung , et al. (2024) In Autoimmunity Reviews 23(12).
Abstract

Immune thrombocytopenia (ITP) is an autoimmune disease characterized by an isolated thrombocytopenia and variable phenotype as some patients suffer no bleeding whilst others have bleeding from mild to severe, which may be fatal. This variability probably reflects the disease's complex pathophysiology; a dysregulated hyperreactive immune effector cell response involving the entire adaptive immune system (e.g. B and T cell subsets) that leads to platelet and megakaryocyte (MK) destruction. It appears that these effector responses are due to a breakdown in immune tolerance, and this is characterized by defects in several immunosuppressive cell types. These include defective T regulatory cells (Tregs), B regulatory cells (Bregs) and... (More)

Immune thrombocytopenia (ITP) is an autoimmune disease characterized by an isolated thrombocytopenia and variable phenotype as some patients suffer no bleeding whilst others have bleeding from mild to severe, which may be fatal. This variability probably reflects the disease's complex pathophysiology; a dysregulated hyperreactive immune effector cell response involving the entire adaptive immune system (e.g. B and T cell subsets) that leads to platelet and megakaryocyte (MK) destruction. It appears that these effector responses are due to a breakdown in immune tolerance, and this is characterized by defects in several immunosuppressive cell types. These include defective T regulatory cells (Tregs), B regulatory cells (Bregs) and Myeloid-derived suppressor cells (MDSC), all of which are all intimately associated with antigen presenting cells (APC) such as dendritic cells (DC). The loss of this immunosuppressive axis allows for the activation of unchecked autoreactive T cells and B cells, leading to the development of autoantibodies and cytotoxic T cells (CTL), which can directly destroy platelets in the periphery and inhibit MK platelet production in the bone marrow (BM). This review will focus on the effector cell mechanisms in ITP and highlight the defective immunosuppressive axis that appears responsible for this platelet-specific immune hyperreactivity.

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type
Contribution to journal
publication status
published
subject
keywords
Autoimmunity, B regulatory cells (Bregs), CD4+ T helper (Th) cells, Cytokines, Cytotoxic T cells (CTL), Immune thrombocytopenia, micro RNAs, Myeloid derived suppressor cells (MDSC), Natural Killer (NK) cells, T regulatory cells (Tregs), Th1 cells, Th17 cell, Th17/Treg ratio, Th22 cells, Th9 cells
in
Autoimmunity Reviews
volume
23
issue
12
article number
103677
publisher
Elsevier
external identifiers
  • pmid:39515406
  • scopus:85208484112
ISSN
1568-9972
DOI
10.1016/j.autrev.2024.103677
language
English
LU publication?
yes
additional info
Publisher Copyright: © 2024
id
78ecdd66-1f05-47fb-8884-2df85ca5dcca
date added to LUP
2025-01-09 09:31:48
date last changed
2025-07-11 00:56:58
@article{78ecdd66-1f05-47fb-8884-2df85ca5dcca,
  abstract     = {{<p>Immune thrombocytopenia (ITP) is an autoimmune disease characterized by an isolated thrombocytopenia and variable phenotype as some patients suffer no bleeding whilst others have bleeding from mild to severe, which may be fatal. This variability probably reflects the disease's complex pathophysiology; a dysregulated hyperreactive immune effector cell response involving the entire adaptive immune system (e.g. B and T cell subsets) that leads to platelet and megakaryocyte (MK) destruction. It appears that these effector responses are due to a breakdown in immune tolerance, and this is characterized by defects in several immunosuppressive cell types. These include defective T regulatory cells (Tregs), B regulatory cells (Bregs) and Myeloid-derived suppressor cells (MDSC), all of which are all intimately associated with antigen presenting cells (APC) such as dendritic cells (DC). The loss of this immunosuppressive axis allows for the activation of unchecked autoreactive T cells and B cells, leading to the development of autoantibodies and cytotoxic T cells (CTL), which can directly destroy platelets in the periphery and inhibit MK platelet production in the bone marrow (BM). This review will focus on the effector cell mechanisms in ITP and highlight the defective immunosuppressive axis that appears responsible for this platelet-specific immune hyperreactivity.</p>}},
  author       = {{Li, Qizhao and Marcoux, Geneviève and Hu, Yuefen and Rebetz, Johan and Guo, Li and Semple, Elisabeth and Provan, Drew and Xu, Shuqian and Hou, Ming and Peng, Jung and Semple, John W.}},
  issn         = {{1568-9972}},
  keywords     = {{Autoimmunity; B regulatory cells (Bregs); CD4+ T helper (Th) cells; Cytokines; Cytotoxic T cells (CTL); Immune thrombocytopenia; micro RNAs; Myeloid derived suppressor cells (MDSC); Natural Killer (NK) cells; T regulatory cells (Tregs); Th1 cells; Th17 cell; Th17/Treg ratio; Th22 cells; Th9 cells}},
  language     = {{eng}},
  number       = {{12}},
  publisher    = {{Elsevier}},
  series       = {{Autoimmunity Reviews}},
  title        = {{Autoimmune effector mechanisms associated with a defective immunosuppressive axis in immune thrombocytopenia (ITP)}},
  url          = {{http://dx.doi.org/10.1016/j.autrev.2024.103677}},
  doi          = {{10.1016/j.autrev.2024.103677}},
  volume       = {{23}},
  year         = {{2024}},
}