Background: Immune thrombocytopenia (ITP) is caused by immune-mediated platelet destruction and reduced platelet production. The aim of this review article is to provide an updated overview of pathophysiology and new therapeutic modalities in ITP.
Material and methods: The article is based on literature identified through a non-systematic search in PubMed and our own clinical experience.
Results: ITP is diagnosed in patients with platelet count < 100 × 10(9)/l after excluding other causes of thrombocytopenia. Anti-platelet autoantibodies are important in the platelet destruction mechanism, but other important mechanisms have been identified in recent years. Patients with very low platelet count < 30 × 10(9)/l are particularly susceptible to bleeding complications. The goal of treatment so far has been to increase the platelet count to a level that reduces the risk of serious bleeding. Thrombopoietin receptor agonists are new therapeutic agents that target the thrombopoietin receptor to increase platelet production. These drugs are shown to be effective in treatment of ITP.
Interpretation: New knowledge about pathophysiological mechanisms, such as sub-optimal platelet production in ITP, has led to the development of new therapeutic options which focus on stimulation of platelet production.