Treatment of Nodal Lymphomas of T Follicular Helper Cell Origin

The 2016 World Health Organization (WHO) classification recognized nodal lymphomas of T follicular helper (TFH) cell origin as a distinct subset of peripheral T-cell lymphoma (PTCL). Unified by a CD4+ TFH cell origin, this subset includes:[1]

AITL is the most common of these entities and is the second-most common subtype of PTCL.[2-5] Patients often present with profound lymphadenopathy, fever, night sweats, weight loss, skin rash, a positive Coombs test, and polyclonal hypergammaglobulinemia.[6] Opportunistic infections are frequent because of an underlying immune deficiency. B-cell Epstein-Barr virus genomes are detected in most affected patients.[7] For more information about weight loss, see Nutrition in Cancer Care and for more information about skin rash, see Pruritus.

The remaining subtypes of nodal lymphomas of TFH cell origin, including F-PTCL and nodal PTCL with TFH phenotype, are derived from the same cell of origin. However, unlike AITL, these subtypes are not associated with hypervascularity on nodal biopsy and often lack the hyperinflammatory symptoms characteristic of AITL.[1]