Immune thrombocytopenic purpura.
Immune thrombocytopenic purpura (ITP) is an autoantibody-mediated thrombocytopenic disorder in which accelerated destruction of platelets occurs; platelet production may also be impaired by these antibodies. ITP is characterized by mucocutaneous bleeding. Rarely, more severe hemorrhages, such as intracranial hemorrhage, may occur. Traditional therapies, such as steroids, immunoglobulin therapy, and splenectomy, generally reduce peripheral destruction of platelets. More recently, with a better understanding of the immunopathologic mechanisms underlying thrombocytopenia, several new treatments have been developed, including thrombopoietic agents, specific inhibitors of Fcgamma receptor (FcgammaR) signaling, and B-cell depletion therapies. This article outlines current understanding of the epidemiology, etiology, diagnosis, and treatment of ITP. The focus is on recent pathophysiologic insights and areas of potential controversy in which studies are ongoing.
Division of Pediatric Hematology-Oncology, Weill-Cornell Medical College of Cornell University, 515 East 71st Street, S-724, New York, NY 10021, USA.
Bethan
B
Psaila
James B
JB
Bussel
D003131:Combined Modality Therapy; D004358:Drug Therapy; D006801:Humans; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000175:diagnosis / Q000209:etiology / Q000628:therapy