A healthy 70-year-old man presented to medical attention after noticing a progressively enlarging right neck mass over several weeks, followed by the appearance of a similar mass on the left side. PET-CT revealed extensive FDG-avid nodal disease with bilateral cervical conglomerate involving bilateral cervical and multiple lymph node regions above and below the diaphragm. While awaiting repeat biopsy, he developed shortness of breath and presented to the emergency department. Core needle biopsy demonstrated effacement of nodal architecture by large neoplastic cells that were marked as weak CD3-positive T-cells, of the CD4 subset, co-expressing CD5, CD7, TCR-βF1, and CD30 (20%), and positive for the T follicular helper cell markers BCL6 and PD-1 (CD279). The cells were negative for CXCL13 and ICOS. The findings were reported as consistent with T follicular helper cell lymphoma, NOS (TFH). He presented to Mayo Clinic, Rochester, for consultation where due to his high disease burden and severe constitutional symptoms, he was admitted for expedited staging and initiation of systemic therapy with BV-CHP. After a single treatment, his bulky cervical lymphadenopathy markedly regressed, and PET-CT after two cycles showed an almost complete response. He completed six cycles of BV-CHP, achieving complete remission. Post-induction strategies, including autologous stem cell transplantation (ASCT) or observation were reviewed in detail. Although initially interested in transplant, the patient elected to pursue clinical trial enrollment (EA4232, a phase III trial comparing ASCT to observation) after learning of the lack of randomized data supporting a clear benefit from transplant and the potential for treatment-related toxicity.
