Evaluation and management of angioimmunoblastic T-cell lymphoma: a review of current approaches and future strategies

Clin Adv Hematol Oncol. 2008 Dec;6(12):899-909.


Angioimmunoblastic T-cell lymphoma (AITL) is a rare and complex lymphoproliferative disorder, clinically characterized by widespread lymphadenopathy, extranodal disease, immune-mediated hemolysis, and polyclonal hypergammaglobulinemia. Significant progress has been made in the understanding of AITL since its recognition as a clonal T-cell disorder with associated deregulation of B-cells and endothelial cells within a unique malignant microenvironment. However, as the responses to conventional chemotherapy have not been durable, prognosis with current treatment approaches has remained dismal. Here we review the clinical presentation, prognosis, and management of patients with AITL. We discuss recent developments in the understanding of the pathogenesis of AITL at a cellular and molecular level, including the implication of the follicular helper T-cell as the corresponding cell of origin, the roles of Epstein-Barr virus, B-cell deregulation, angiogenesis, and other signaling pathways in AITL, and the therapeutic implications of these findings. Finally, we discuss recent clinical trials and novel treatment approaches in the management of patients with AITL.

Publication types

  • Review

MeSH terms

  • Angiogenesis Inhibitors / therapeutic use
  • Animals
  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Monoclonal, Humanized
  • Antibodies, Monoclonal, Murine-Derived
  • Antineoplastic Agents / therapeutic use
  • B-Lymphocytes / immunology
  • B-Lymphocytes / pathology
  • Bevacizumab
  • Cyclosporine / therapeutic use
  • Cytogenetics
  • Drug Therapy
  • Epstein-Barr Virus Infections / pathology
  • Humans
  • Immunoblastic Lymphadenopathy / diagnosis*
  • Immunoblastic Lymphadenopathy / drug therapy
  • Immunoblastic Lymphadenopathy / physiopathology
  • Immunoblastic Lymphadenopathy / therapy*
  • Immunosuppressive Agents / therapeutic use
  • Lymphoma, T-Cell, Peripheral / diagnosis*
  • Lymphoma, T-Cell, Peripheral / drug therapy
  • Lymphoma, T-Cell, Peripheral / physiopathology
  • Lymphoma, T-Cell, Peripheral / therapy*
  • Rituximab
  • T-Lymphocytes / immunology
  • T-Lymphocytes / pathology
  • Transplantation


  • Angiogenesis Inhibitors
  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Humanized
  • Antibodies, Monoclonal, Murine-Derived
  • Antineoplastic Agents
  • Immunosuppressive Agents
  • Bevacizumab
  • Rituximab
  • Cyclosporine