title: Immune thrombocytopenia and B-cell-activating factor/a proliferation-inducing ligand.
abstract: Primary immune thrombocytopenia (ITP) is an organ-specific autoimmune disorder characterized by autoantibody-mediated enhanced platelet destruction and dysmegakaryocytopoiesis. B cells have been demonstrated to play critical roles in the pathophysiology of ITP. B-cell-activating factor (BAFF) and a proliferation-inducing ligand (APRIL) are crucial cytokines supporting survival and differentiation of B cells, and dysregulation of BAFF/APRIL is involved in the pathogenesis of B-cell related autoimmune diseases including ITP. Currently ongoing clinical trials using BAFF and/or APRIL-blocking agents have yielded positive results in human systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA), further confirming the pathological role of BAFF/APRIL in autoimmunity. This review will describe the function of BAFF/APRIL and address the feasibility of BAFF/APRIL inhibition in the management of ITP.
authors.affiliation: Department of Hematology, Qilu Hospital, Shandong University, Jinan, PR China.
authors.forename: Xin-guang
authors.initials: XG
authors.lastname: Liu
authors.forename: Ming
authors.initials: M
authors.lastname: Hou
mesh_terms: D053264:B-Cell Activating Factor / Q000276:immunology*; D001402:B-Lymphocytes / Q000276:immunology*; D048708:Cell Growth Processes / Q000276:immunology; D006801:Humans; D008024:Ligands; D013921:Thrombocytopenia / Q000276:immunology*
chemical_list: D053264:B-Cell Activating Factor; D008024:Ligands