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Review
. 2024 Apr 3;16(2):204-219.
doi: 10.3390/hematolrep16020021.

Pathophysiology, Clinical Manifestations and Diagnosis of Immune Thrombocytopenia: Contextualization from a Historical Perspective

Daniel Martínez-Carballeira  1   2 , Ángel Bernardo  1   2 , Alberto Caro  1   2 , Inmaculada Soto  1   2 , Laura Gutiérrez  2   3
Affiliations
Review

Pathophysiology, Clinical Manifestations and Diagnosis of Immune Thrombocytopenia: Contextualization from a Historical Perspective

Daniel Martínez-Carballeira et al. Hematol Rep. .

Abstract

Immune thrombocytopenia (ITP) is an autoimmune disease characterized by an isolated decrease in the platelet count and an increased risk of bleeding. The pathogenesis is complex, affecting multiple components of the immune system and causing both peripheral destruction of platelets and impaired central megakaryopoiesis and platelet production in the bone marrow. Here, we intend to contextualize the current knowledge on the pathophysiology, terminology, epidemiology, clinical manifestations, diagnosis, and prognosis of ITP from a historical perspective and the first references to the never-stopping garnering of knowledge about this entity. We highlight the necessity to better understand ITP in order to be able to provide ITP patients with personalized treatment options, improving disease prognosis and reducing the incidence or frequency of refractoriness.

Keywords: antiplatelet antibodies; clinical manifestations; diagnosis; history; immune thrombocytopenia (ITP); pathogenesis; platelets.

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Conflict of interest statement

Author L.G. is co-founder and CSO of Platelet Biotechnologies S.L. (PlaBiTe). The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Pathophysiology of ITP. Dysfunction of regulatory T cells (Treg) leads to a disruption in the regulation of T helper cell (Th)-mediated B cell activation. B cells, in turn, produce autoantibodies in abundance that lead to opsonization, phagocytosis (by macrophages -MΦ-, and mediated by the spleen tyrosine kinase -Syk-) and complement activation, desialylation, and finally destruction of platelets. Autoantibodies further hinder megakaryocyte maturation, and autoreactive cytotoxic T cells (Tc) destroy megakaryocytes and platelets. HSC, hematopoietic stem cell; MK-blast, megakaryoblast; MK, megakaryocyte; PLT, platelet; AMR, Ashwell–Morell receptor. The figure was generated using Servier Medical Art, provided by Servier, licensed under a Creative Commons Attribution 3.0 unported license (https://creativecommons.org/licenses/by/3.0/). Image adapted from: Singh, A.; et al. J. Clin. Med. 2021, 10 (4): 789. [60].
See this image and copyright information in PMC

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