Immune dysregulation in immune thrombocytopenia

Abstract

Immune thrombocytopenia (ITP) is a bleeding disorder characterized by low platelet counts due to decreased platelet production as well as increased platelet destruction by autoimmune mechanisms. A shift toward Th1 and possibly Th17 cells together with impaired regulatory compartment, including T-regulatory (Tregs) and B-regulatory (Bregs) cells, have been reported, suggesting a generalized immune dysregulation in ITP. Interestingly, several treatments including the use of thrombopoietic agents appear to be associated with improvement in the regulatory compartment. Understanding how Th1/Th17/Treg differentiation and expansion are controlled is central to uncovering how autoimmunity may be sustained in chronic ITP and reversed following response to therapy. In this review, we will summarize the recent findings on the state of the Breg and Treg compartments in ITP, the role of monocyte subsets in the control of Th/Treg expansion, and our working model of how the regulatory compartment may impact response to treatment and the means by which this information may guide therapy in ITP patients in the future.