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Idiopathic thrombocytopenic purpura: Current concepts in pathophysiology and management

Journal: Thrombosis and Haemostasis
ISSN: 0340-6245
DOI: http://dx.doi.org/10.1160/TH07-08-0513
Issue: 2008: 99/1 (Jan) pp. 1-248
Pages: 4-13

Idiopathic thrombocytopenic purpura: Current concepts in pathophysiology and management

Roberto Stasi1, Maria Laura Evangelista1, Elisa Stipa2, Francesco Buccisano3, Adriano Venditti3, Sergio Amadori3
1Department of Medical Sciences, Ospedale „Regina Apostolorum“, Albano Laziale, Italy; 2Division of Hematology, Ospedale S. Eugenio, Rome, Italy; 3Department of Hematology, “Tor Vergata” University Hospital, Rome, Italy

Summary

Idiopathic thrombocytopenic purpura (ITP) is characterized by a low platelet count, which is the result of both increased platelet destruction and insufficient platelet production. Although the development of autoantibodies against platelet glycoproteins remains central in the pathophysiology of ITP, several abnormalities involving the cellular mechanisms of immune modulation have been identified. Conventional treatments for ITP aim at reducing platelet destruction, either by immunosuppression or splenectomy.Two new thrombopoietic agents,AMG 531 and eltrombopag, have been used in clinical trials to stimulate platelet production in ITP patients not responsive to standard treatments. These new molecules bear no structural resemblance to thrombopoietin, but still bind and activate the thrombopoietin receptor.This review will focus on the pathophysiology and treatment of ITP in adults, highlighting recent advances in both fields.

Keywords

Thrombocytopenia, Platelet immunology, immunity

DOI

http://dx.doi.org/10.1160/TH07-08-0513

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