A 69-year-old male presented with a 5-month history of pruritus and lymphocytosis. The exam revealed a 5 cm purple nodule on the left axilla, and pink papules and plaques on the back. Punch biopsies showed dense dermal infiltrates of atypical CD4+, PD1+, and GATA3+ T-cells with intraepidermal and follicular involvement. A monoclonal T-cell receptor gene rearrangement was detected. Flow cytometry confirmed aberrant CD4+ T-cells with a CD4:CD8 ratio of 156:1. FISH was negative for TCL1A/TRAD rearrangement. Bone marrow biopsy revealed 20-30% infiltration of CD4+ T-cell lymphoma. PET-CT showed diffuse FDG-avid lymphadenopathy and cutaneous involvement in the left axilla. The patient was diagnosed with stage IV peripheral T-cell lymphoma (PTCL).
He began treatment with BV-CHEP. His course was complicated by urinary retention, night sweats, and worsening lymphocytosis. His skin showed minimal response, and a punch biopsy confirmed TP53-mutated CD4+ T-cell lymphoma. Romidepsin/duvelisib therapy was initiated but led to transaminitis and a progressive erythematous rash. He then received two cycles of alemtuzumab with clearance of the blood compartment and completed TSEB, 1200 cGy over 12 days, with clearance of the skin. He resumed alemtuzumab and added pentostatin, with the goal of an allogeneic stem cell transplant (alloSCT), with a PET scan showing no active lymphoma.
This case demonstrates the challenge in treating TP53-mutated PTCL due to its high risk of refractory disease and the difficulty achieving CR in the compartments of skin, blood, and lymph nodes. Patients who are in CR at the time of transplant have better overall survival and progression-free survival.
