An update on the pathophysiology of immune thrombocytopenia
Semple, John W. LU ; Rebetz, Johan LU
; Maouia, Amal
LU
and Kapur, Rick
LU
(2020)
In Current Opinion in Hematology
27(6).
p.423-429
- Abstract
Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder mediated by antiplatelet autoantibodies and antigen-specific T cells that either destroy platelets peripherally in the spleen or impair platelet production in the bone marrow. There have been a plethora of publications relating to the pathophysiology of ITP and since January of 2019, at least 50 papers have been published on ITP pathophysiology. PURPOSE OF REVIEW: To summarize the literature relating to the pathophysiology of ITP including the working mechanisms of therapies, T-cell and B-cell physiology, protein/RNA/DNA biochemistry, and animal models in an attempt to unify the perceived abnormal immune processes. RECENT FINDINGS: The most recent pathophysiologic... (More)
Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder mediated by antiplatelet autoantibodies and antigen-specific T cells that either destroy platelets peripherally in the spleen or impair platelet production in the bone marrow. There have been a plethora of publications relating to the pathophysiology of ITP and since January of 2019, at least 50 papers have been published on ITP pathophysiology. PURPOSE OF REVIEW: To summarize the literature relating to the pathophysiology of ITP including the working mechanisms of therapies, T-cell and B-cell physiology, protein/RNA/DNA biochemistry, and animal models in an attempt to unify the perceived abnormal immune processes. RECENT FINDINGS: The most recent pathophysiologic irregularities associated with ITP relate to abnormal T-cell responses, particularly, defective T regulatory cell activity and how therapeutics can restore these responses. The robust literature on T cells in ITP points to the notion that ITP is a disease initiated by faulty self-tolerance mechanisms very much like that of other organ-specific autoimmune diseases. There is also a large literature on new and existing animal models of ITP and these will be discussed. It appears that understanding how to specifically modulate T cells in patients with ITP will undoubtedly lead to effective antigen-specific therapeutics. CONCLUSIONS: ITP is predominately a T cell disorder which leads to a breakdown in self tolerance mechanisms and allows for the generation of anti-platelet autoantibodies and T cells. Novel therapeutics that target T cells may be the most effective way to perhaps cure this disorder.
(Less)
- author
- Semple, John W.
LU
; Rebetz, Johan
LU
; Maouia, Amal
LU
and Kapur, Rick
LU
- organization
- publishing date
- 2020
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Current Opinion in Hematology
- volume
- 27
- issue
- 6
- pages
- 7 pages
- publisher
- Lippincott Williams & Wilkins
- external identifiers
-
- scopus:85092681428
- pmid:32868673
- ISSN
- 1531-7048
- DOI
- 10.1097/MOH.0000000000000612
- language
- English
- LU publication?
- yes
- id
- 6ebf19b2-8a47-41ad-9274-d27f614fce78
- date added to LUP
- 2020-11-05 13:37:19
- date last changed
- 2024-11-15 16:18:22
@article{6ebf19b2-8a47-41ad-9274-d27f614fce78,
abstract = {{<p> Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder mediated by antiplatelet autoantibodies and antigen-specific T cells that either destroy platelets peripherally in the spleen or impair platelet production in the bone marrow. There have been a plethora of publications relating to the pathophysiology of ITP and since January of 2019, at least 50 papers have been published on ITP pathophysiology. PURPOSE OF REVIEW: To summarize the literature relating to the pathophysiology of ITP including the working mechanisms of therapies, T-cell and B-cell physiology, protein/RNA/DNA biochemistry, and animal models in an attempt to unify the perceived abnormal immune processes. RECENT FINDINGS: The most recent pathophysiologic irregularities associated with ITP relate to abnormal T-cell responses, particularly, defective T regulatory cell activity and how therapeutics can restore these responses. The robust literature on T cells in ITP points to the notion that ITP is a disease initiated by faulty self-tolerance mechanisms very much like that of other organ-specific autoimmune diseases. There is also a large literature on new and existing animal models of ITP and these will be discussed. It appears that understanding how to specifically modulate T cells in patients with ITP will undoubtedly lead to effective antigen-specific therapeutics. CONCLUSIONS: ITP is predominately a T cell disorder which leads to a breakdown in self tolerance mechanisms and allows for the generation of anti-platelet autoantibodies and T cells. Novel therapeutics that target T cells may be the most effective way to perhaps cure this disorder.</p>}},
author = {{Semple, John W. and Rebetz, Johan and Maouia, Amal and Kapur, Rick}},
issn = {{1531-7048}},
language = {{eng}},
number = {{6}},
pages = {{423--429}},
publisher = {{Lippincott Williams & Wilkins}},
series = {{Current Opinion in Hematology}},
title = {{An update on the pathophysiology of immune thrombocytopenia}},
url = {{http://dx.doi.org/10.1097/MOH.0000000000000612}},
doi = {{10.1097/MOH.0000000000000612}},
volume = {{27}},
year = {{2020}},
}