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J. Clin. Med. 2017, 6(2), 16; doi:10.3390/jcm6020016

Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP)

1,2
,
1,2,3
and
1,2,3,4,5,*
1
Keenan Research Centre for Biomedical Science, St. Michael’s Hospital, Toronto, ON M5B 1W8, Canada
2
The Toronto Platelet Immunobiology Group, St. Michael’s Hospital, Toronto, ON M5B 1W8, Canada
3
Canadian Blood Services, Toronto, ON M5B 1W8, Canada
4
Department of Pharmacology, Medicine, and Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON M5B 1W8, Canada
5
Division of Hematology and Transfusion Medicine, Lund University, 221 84 Lund, Sweden
*
Author to whom correspondence should be addressed.
Received: 28 December 2016 / Accepted: 4 February 2017 / Published: 9 February 2017
(This article belongs to the Special Issue Thrombocytopenia and ITP: Causes, Symptoms, and Treatment)
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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. 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. View Full-Text
Keywords: immune thrombocytopenia (ITP); autoimmunity; T cells; B cells; platelets immune thrombocytopenia (ITP); autoimmunity; T cells; B cells; platelets
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Zufferey, A.; Kapur, R.; Semple, J.W. Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP). J. Clin. Med. 2017, 6, 16.

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