Management of Angioimmunoblastic T-Cell Lymphoma (AITL) and other T Follicular Helper Cell lymphomas (TFH PTCL)

Abstract

Despite the remarkable improvements in the treatment and outcome of patients with aggressive B-cell lymphoma, the peripheral T-cell lymphomas (PTCL) continue to carry a poor prognosis with the presently available treatment options. The PTCL are very rare diseases that account for only 10,000 to 15,000 new cases per year in the United States. The World Health Organization's 2016 classification describes 29 distinct subtypes of PTCL, thus making these both rate and incredibly heterogenous. The 2 most common forms of PTCL, for example, peripheral T-cell lymphoma-not otherwise specified and angioimmunoblastic T-cell lymphoma , have an incidence of only 2500 and 1800 cases per year respectively, in the United States.

Keywords: Angioimmunoblastic T-cell lymphoma (AITL); Epigenetic Modifiers; Nodal PTCL with T Follicular Helper Phenotype (PTCL TFH).