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 -15,000 new cases per year in the US. The World Health Organization's 2016 classification describes 29 distinct subtypes of PTCL, thus making these both rate and incredibly heterogenous. The two most common forms of PTCL, e.g. peripheral T-cell lymphoma-not otherwise specified (PTCL-NOS) and angioimmunoblastic T-cell lymphoma (AITL), have an incidence of only 2500 and 1800 cases per year respectively, in the U.S.