The ITP syndrome: Pathogenic and clinical diversity Review uri icon


MeSH Major

  • Purpura, Thrombocytopenic, Idiopathic


  • Immune thrombocytopenia (ITP) is mediated by platelet autoantibodies that accelerate platelet destruction and inhibit their production. Most cases are considered idiopathic, whereas others are secondary to coexisting conditions. Insights from secondary forms suggest that the proclivity to develop platelet-reactive antibodies arises through diverse mechanisms. Variability in natural history and response to therapy suggests that primary ITP is also heterogeneous. Certain cases may be secondary to persistent, sometimes inapparent, infections, accompanied by coexisting antibodies that influence outcome. Alternatively, underlying immune deficiencies may emerge. In addition, environmental and genetic factors may impact platelet turnover, propensity to bleed, and response to ITP-directed therapy. We review the pathophysiology of several common secondary forms of ITP. We suggest that primary ITP is also best thought of as an autoimmune syndrome. Better understanding of pathogenesis and tolerance checkpoint defects leading to autoantibody formation may facilitate patient-specific approaches to diagnosis and management.

publication date

  • November 19, 2009



  • Review



  • eng

PubMed Central ID

  • PMC2710913

Digital Object Identifier (DOI)

  • 10.1182/blood-2009-01-129155

PubMed ID

  • 19395674

Additional Document Info

start page

  • 6511

end page

  • 21


  • 113


  • 26