title: Multi-dysfunctional pathophysiology in ITP.
abstract: Idiopathic thrombocytopenic purpura (ITP) is an organ-specific autoimmune disorder characterized by a low platelet count and mucocutaneous bleeding. The decrease of platelets is caused by increased autoantibodies against self-antigens, particularly IgG antibodies against GPIIb/IIIa. The production of these autoantibodies by B cells depends on a number of cellular mechanisms that form a network of modulation, with T cells playing a pivotal role in pathophysiology. Delineation of the dysfunction of cellular immunity has recently been attempted. This review will focus on these recent advances applicable to ITP and to highlight how these may translate into novel approaches to treatment in the future. Multi-dysfunction in these networks may include a failure of self-antigen recognition and tolerance, involvement of abnormal cell surface molecules, altered Th1/Th2 cytokine profiles, impaired megakaryocytopoiesis and impaired cell-mediated cytotoxicity. In ITP, multi-step dysfunctions in these networks may take place that finally lead to the occurrence of the disease. Therefore, unveiling these dysfunctions is vital in understanding the pathophysiology of ITP and will finally lead to the development of new therapies to fight the disease.
authors.affiliation: State Key Laboratory of Experimental Hematology, Institute of Hematology and Hospital of Blood Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, 288 Nanjing Road, Tianjin 300020, PR China.
authors.forename: Bin
authors.initials: B
authors.lastname: Zhou
authors.forename: Hui
authors.initials: H
authors.lastname: Zhao
authors.forename: Ren Chi
authors.initials: RC
authors.lastname: Yang
authors.forename: Zhong Chao
authors.initials: ZC
authors.lastname: Han
mesh_terms: D002450:Cell Communication; D016207:Cytokines / Q000276:immunology; D006801:Humans; D007108:Immune Tolerance; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000209:etiology / Q000276:immunology*
chemical_list: D016207:Cytokines