Introduction: Adult T-cell leukemia/lymphoma (ATLL) is a rare, aggressive T-cell neoplasm linked to chronic HTLV-1 infection. In non-endemic regions like the U.S., affected patients are primarily of Afro-Caribbean and Hispanic descent. Real-world data on treatment outcomes remain limited.
Methods: We retrospectively analyzed 51 patients with confirmed ATLL treated at Moffitt Cancer Center (2006–2024). Diagnosis required HTLV-1 positivity and hematopathologist review. Subtypes included acute (49%), lymphomatous (47%), and chronic (4%). Median age was 54 years (range, 31–85); 78% were Black, and 63% were of Caribbean or U.S. origin. Nearly half presented to us with relapsed or refractory disease. Misdiagnosis occurred in 14% (PTCL-NOS or CTCL without HTLV-1 testing). High-risk features were common: LDH >2× ULN (67%) and hypercalcemia (46%).
Results: Median OS for aggressive subtypes was 10.3 months; 2-year OS was 14%. CHOP or CHOP-like therapy was used in 36 patients (73%) with 33% ORR (30% CR). Responses were transient unless followed by allogeneic transplant (alloHSCT). AZT+IFN was used in 6 patients (12%) with 33% ORR (all PR); 4 patients with acute subtype receiving AZT+IFN survived long term, particularly when followed by mogamulizumab or alloHSCT. Only 8 patients (15.6%) underwent alloHSCT; 3 (37%) remain alive without disease.
Conclusion: Despite aggressive therapy, outcomes remain poor. However, durable responses in a subset of acute ATLL patients suggest potential benefit from early AZT+IFN followed by immune-based consolidation. These findings support further evaluation of AZT+IFN as part of initial therapy in selected patients.
