[Immune thrombocytopenic purpura]

ABSTRACT

It is characterized by a low platelet count < 150000/mm[3], which is the result of both increased platelet destruction and insufficient platelet production. In this paper, we will focus on current aspects of geoepidemiology, pathophysiology, diagnosis and management of primary immune thrombocytopenia in adult. The development of autoantibodies against platelet glycoproteins remains central in the pathophysiology of immune thrombocytopenic purpura, several abnormalities involving the cellular mechanisms of immune modulation have been identified. The goal treatment is to raise the platelet count to high enough levels to prevent bleeding. Corticosteroids, intravenous immune globulin, and splenectomy remain mainstays of treatment; however, newer therapies including rituximab and the thrombopoietin receptor agonists are remodeling conventional treatment algorithms. Effective treatments are aimed at different steps in the pathophysiologic process including the reduction of autoantibody production, interference with FcR uptake and signaling, suppression of B and T cells, and increase in TPO activity. The development of biologic therapies, particularly anti-CD20 and the emergence of new drug to increase platelet production rather than modulating the immune response, however, may radically change the management of immune thrombocytopenic purpura and make the information rarest of splenectomy