Angioimmunoblastic T-Cell Lymphoma Management

Angioimmunoblastic T-cell lymphoma (AITL) is a frequent subtype of peripheral T-cell lymphoma (PTCL) that is clinically characterized by generalized lymphadenopathy, extranodal involvement, advanced stage at presentation, hypergammaglobulinemia, and significant immune dysregulation resulting in infections as the most common cause of death. Recent advances in pathobiology of AITL have improved our understanding of it as a clonal T-cell disorder and of its effect on B cells in the tumor microenvironment. Reponses to first-line therapies have largely been dismal. In this review, we discuss the clinical features, pathobiology, prognostic models, standard therapy, and newer therapeutic agents used and their implications for the future. (C) 2014 Elsevier Inc. All rights reserved.