Angioimmunoblastic T-Cell Lymphoma: Clinical Aspects and Recent Advances in Biology and Therapy


  • Bernardo Garicochea Centro de Oncologia, Hospital Sírio-Libanês, Unidade Bela Vista, São Paulo, Brazi
  • Alessandro Igor Cavalcanti Leal Centro de Oncologia, Hospital Sírio-Libanês, Unidade Brasília, Brasília, Brazi
  • Fernando Sérgio Blumm Ferreira Centro de Oncologia, Hospital Sírio-Libanês, Unidade Brasília, Brasília, Brazi
  • Volney Assis Lara Vilela Centro de Oncologia, Hospital Sírio-Libanês, Unidade Brasília, Brasília, Brazi
  • Alesso Cervantes Sartorelli Laboratório Diagnose, Brasília, Brazil
  • Yana Novis Centro de Oncologia, Hospital Sírio-Libanês, Unidade Bela Vista, São Paulo, Brazi
  • Paulo Marcelo Gehm Hoff Centro de Oncologia, Hospital Sírio-Libanês, Unidade Brasília, Brasília, Brazi



Angioimmunoblastic T-cell lymphoma, T-follicular helper cells, gene expression profiling, TET2 mutation, RHOA GTPase mutation, relapsed disease.


  Angioimmunoblastic T-cell lymphoma (AITL) comprehends 20% of the peripheral T-cell lymphomas (PTCL). Although rare, its clinical features may overlap with many other inflammatory, infectious or neoplastic disorders. Therefore, that patients are often diagnosed with advanced stage disease, which contributes for the disease´s dismal prognosis. The clinical presentation of AITL is frequently an assemblage of symptoms including generalized and painful lymphadenopathy, multiple cutaneous alterations, hypergammaglobulinemia, fever, loss of weight and significant autoimmune phenomena. Recent advances in AITL biology have implicated a cell with T-follicular helper phenotype as the origin of the disorder. This rare type of T lymphocyte has a peculiar capacity of interact with microenviroment, which results in an important production of cytokines, explaining the clinical findings of this type of lymphoma. In addition to its pathologic features, AITL can be distinguished from other T-cell lymphomas based on gene expression arrangement, suggesting that AITL has a uniquebiology. Moreover, somatic mutations in the epigenetic regulators DNMT3A, TET2, IDH2, and, especially, in the multifunctional RHOA GTPase gene, have emerged as very consistent genetic abnormalities in AITL. Considering its low incidence, the development of clinical trials in AITL is a challenging matter. Furthermore, the majority of data available originates from studies that contain other subtypes of PTCL, making prognosis analysis and treatment decision a tough work. In this review, we discuss the biological and clinical aspects of AITL and the alternatives for frontline treatment and the management of relapsed disease.


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How to Cite

Bernardo Garicochea, Alessandro Igor Cavalcanti Leal, Fernando Sérgio Blumm Ferreira, Volney Assis Lara Vilela, Alesso Cervantes Sartorelli, Yana Novis, & Paulo Marcelo Gehm Hoff. (2014). Angioimmunoblastic T-Cell Lymphoma: Clinical Aspects and Recent Advances in Biology and Therapy. Journal of Analytical Oncology, 3(4),  191–200.