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. © 2013 Elsevier Inc.