Cellular immune dysregulation in the pathogenesis of immune thrombocytopenia

Abstract

Immune thrombocytopenia (ITP) is an acquired autoimmune hemorrhagic disease characterized by immune-mediated increased platelet destruction and decreased platelet production, resulting from immune intolerance to autoantigen. The pathogenesis of ITP remains unclear, although dysfunction of T and B lymphocytes has been shown to be involved in the pathogenesis of ITP. More recently, it is found that dendritic cells, natural killer, and myeloid-derived suppressor cells also play an important role in ITP. Elucidating its pathogenesis is expected to provide novel channels for the targeted therapy of ITP. This article will review the role of different immune cells in ITP.

Pathogenesis of refractory ITP: Overview

“…56 Fourth, alterations in dendritic cell, mesenchymal stem cell, natural killer (NK) cell and myeloid suppressor cell functions that would be predicted to enhance immune responses, have also been reported in small studies, but not as yet related specifically to rITP. [61][62][63][64] Fifth, consistent with these alterations in immune cell expression, a pro-inflammatory cytokine profile in the peripheral blood is seen during clinically active disease, characterized by increased IL2, IFNγ, IL17, IL21, IL12/23 and BAFF and reduced IL10, IL-7 and TIMP3. What is needed is to know whether these disturbances precede clinical relapse (if not initial presentation) and which cytokine profiles best distinguish responsive from rITP.…”

“…Fourth, alterations in dendritic cell, mesenchymal stem cell, natural killer (NK) cell and myeloid suppressor cell functions that would be predicted to enhance immune responses, have also been reported in small studies, but not as yet related specifically to rITP 61–64 …”

Primary immune thrombocytopenia in adults: Belgian recommendations for diagnosis and treatment anno 2021 made by the Belgian Hematology Society

“…PATHOGENESIS (Figure 1) [14][15][16][17] New insights in the pathophysiology underlying ITP have taught us that not only accelerated peripheral platelet destruction but also suppression of the production of new platelets can be responsible for the thrombocytopenia. Antibody-loaded platelets bind to macrophages and dendritic cells by the Fc gamma receptors (FcγRs) and are removed, primarily in the spleen.…”

Immune thrombocytopenia: what can the systems biology and systems physiology offer?

“…Therefore, it can be expected that not only humoral immunity contributes to the ITP pathogenesis. Indeed, T-cellular immunity dysregulation ITP was earlier described [50,51]. T-cellular mechanism of ITP is assumed to constitute in T-killers' action against platelet specific antigens, and the T-helpers and T-regulatory cells are also actively involved in the ITP pathogenesis.…”