A 50-year-old male presented with pruritus, generalized lymphadenopathy, thrombocytopenia, and fatigue. PET-CT demonstrated bilateral cervical, axillary, mediastinal, abdominal, and retroperitoneal lymphadenopathy, as well as splenomegaly, hepatomegaly, and diffuse splenic involvement. Excisional lymph-node biopsy confirmed angioimmunoblastic T-cell lymphoma (AITL) with a follicular helper T-cell phenotype (CD30-positive, ALK-negative). Bone-marrow biopsy revealed clonal cytopenia of undetermined significance (CCUS) with DNMT3A mutation but no lymphoma involvement.
Given CD30 expression, first-line treatment with BV-CHP was initiated. After three cycles, the patient exhibited stable disease by Lugano criteria, and therapy was switched to ICE chemotherapy. Peripheral blood stem cells were successfully mobilized. PET-CT after two cycles demonstrated a partial metabolic response, and the patient proceeded to high-dose chemotherapy with LEAM followed by autologous stem-cell transplantation (ASCT).
At day +100 post-ASCT, PET-CT showed disease progression with multiple new FDG-avid lesions. Subsequent therapy with two cycles of DHAP was administered, but PET-CT demonstrated persistent metabolically active disease. Due to treatment-related toxicities and absence of clinical benefit, therapy was transitioned to a GDP. After two cycles, the patient developed liver failure and died at the beginning of cycle three, timeline - Figure 1.
Conclusion:
This case highlights the refractory nature of AITL and the challenge of achieving durable remission despite CD30-directed frontline therapy, intensive salvage regimens, and ASCT consolidation. Disease progression across multiple lines of therapy underscores the need for earlier access to novel agents. Importantly, poor adherence related to alcohol-use disorder and the unavailability of approved therapies such as belinostat and duvelisib negatively influenced clinical outcome.
