Follicular T-cell lymphoma: a short review with brief discussion of other nodal lymphomas/lymphoproliferative disorders of T-follicular helper cell origin

Follicular peripheral T-cell lymphoma (F-PTCL) is a newly recognized provisional entity in the 2017 World Health Organization Classification revision of hematolymphoid neoplasms. It rare and under-recognized manifestation that associated with typically aggressive clinical course. Cases feature predominantly follicular or perifollicular growth pattern exhibit strong consistent expression T-follicular helper (TFH) markers. These cases distinctly lack histologic features commonly angioimmunoblastic lymphoma, such as an polymorphous infiltrate, proliferation high endothelial venules expanded dendritic meshworks. A relatively specific t(5;9)(q33;q22) rearrangement, leading to ITK-SYK fusion seen about 20% cases. Herein, we summarize key F-PTCL briefly review evolving relationship other nodal lymphomas/lymphoproliferative disorders TFH-derivation.