Abstract
S74 Caribbean, and rarely encountered within the United States. ATLL is sub-divided in 5 categories: acute, lymphomatous, chronic, smoldering, and Hodgkin like. Much is still to be established with this rare disease including a standard of care. The high prevalence of patients with HTLV-1 ATLL in the Caribbean places our institution within close proximity as opposed to other areas of the United States. Methods: We identified HTLV-1 ATLL patients treated at Moffitt Cancer Center (MCC). Records were reviewed for demographics, disease features, and outcomes. Results: Ten ATLL patients were treated at MCC over the past 5 years. The median age was 56 years. Six patients were females and 7 were originally from the Caribbean. Seven patients were leukemic subtype, 9 patients had lymphadenopathy, 7 patients had bone marrow involvement, 5 had skin rash, 1 patient had bone disease, and none of the patients had hepatosplenomegaly, nor CNS disease. The mean hemoglobin was 12 g/dl, WBC 17/mcl, and platelets 266/mcl. All patients had hypercalcemia (mean serum calcium 11.7mg/dl), the mean serum LDH was 1238 U/L. By immunophenotype, all cases were CD3+, 90% CD5+, 80% CD25+, and 10% CD30+. Seven patients received CHOP regimen as first line therapy, and 2 patients interferon/zidovudine, 1 patient had VCAP-AMP-VECP. Only 1 patient had complete response (CR) to first line therapy, 4 had partial responses, and 3 had progressive disease. Subsequent therapy included interferon/zidovudine in 2 patients, HyperCVAD 2 patients, ESHAP 2 patients, and ICE, IGEV, pralatrexate, and denileukin difitox in 1 patient each. Three patients proceeded to allogeneic stem cell transplant (ASCT). The median overall survival (OS) was 51 months (mo). The median OS was 88 mo among those who underwent ASCT versus 34 mo for those who did not. Conclusion: HTLV-1 ATLL is associated with poor outcomes. Response to CHOP chemotherapy is poor. Best outcomes were achieved if patients proceeded to ASCT in CR1.
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