The Centenary of Immune Thrombocytopenia-Part 2: Revising Diagnostic and Therapeutic Approach

Abstract

Primary immune thrombocytopenia (ITP) is the most common cause of thrombocytopenia in children and adolescents and can be considered as a paradigmatic model of autoimmune disease. This second part of our review describes the clinical presentation of ITP, the diagnostic approach and overviews the current therapeutic strategies. Interestingly, it suggests an algorithm useful for differential diagnosis, a crucial process to exclude secondary forms of immune thrombocytopenia (IT) and non-immune thrombocytopenia (non-IT), which require a different therapeutic management. Advances in understanding the pathogenesis led to new therapeutic targets, as thrombopoietin receptor agonists, whose role in treatment of ITP will be discussed in this work.

Keywords: chronic thrombocytopenia; conventional therapy; diagnostic algorithm; differential diagnosis; immune thrombocytopenia; new therapeutic targets.

Figure 1

Algorithm for differential diagnosis of immune thrombocytopenia, composed by four progressive steps. *Testing for Epstein–Barr virus (EBV), cytomegalovirus (CMV), and HCV is recommended if not done in previous step. **Helycobacter pylori testing should be performed in high prevalence area or clinical suspect.

Figure 2

(Left) The new therapeutic targets identified by the study of pathogenesis of immune thrombocytopenia. (Right) Corresponding drug classes.