Imbalanced immune homeostasis in immune thrombocytopenia

Semin Hematol. 2016 Apr;53 Suppl 1(Suppl 1):S16-9. doi: 10.1053/j.seminhematol.2016.04.006. Epub 2016 Apr 7.

Abstract

Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder resulting from low platelet counts caused by inadequate production as well as increased destruction by autoimmune mechanisms. As with other autoimmune disorders, chronic ITP is characterized by perturbations of immune homeostasis with hyperactivated effector cells as well as defective regulatory arm of the adaptive immune system, which will be reviewed here. Interestingly, some ITP treatments are associated with restoring the regulatory imbalance, although it remains unclear whether the immune system is redirected to a state of tolerance once treatment is discontinued. Understanding the mechanisms that result in breakdown of immune homeostasis in ITP will help to identify novel pathways for restoring tolerance and inhibiting effector cell responses. This information can then be translated into developing therapies for averting autoimmunity not only in ITP but also many autoimmune disorders.

Keywords: Immune dysregulation; Immune thrombocytopenia; Thrombopoietin.

MeSH terms

  • Homeostasis / immunology*
  • Humans
  • Purpura, Thrombocytopenic, Idiopathic / immunology*