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Atypical Immunophenotype Predicts Worse Prognosis in Adult T-Cell Lymphoma/Leukemia: A Retrospective Study of 63 Caribbean Patients at a New York City Tertiary Center
Bachar Samra1, Edwin Chiu1, Bo Lin2, Eric Tam1, Babak Baseri1, Iuliana Shapira1, Jason Gonsky3, Robert Lewis3, Gurinder Sidhu1, Ahmed Sawas4, Evelyn Taiwo3. 1Hematology/Oncology, SUNY Downstate, Brooklyn, New York, United States, 2Pathology, SUNY Downstate, Brooklyn, New York, United States, 3Hematology/Oncology, Kings County Hospital, Brooklyn, New York, United States, 4Center for Lymphoid Malignancies, Columbia University Medical Center, New York, New York, United States

Purpose of Study Adult T-Cell Lymphoma/Leukemia (ATLL) is a rare and aggressive HTLV-1 related peripheral T-cell lymphoma that occurs predominantly in Japan and the Caribbean basin. Acute (A) and lymphomatous (L) subtypes are the most aggressive forms with a median overall survival (OS) of 6-12 months despite chemotherapy. Our primary objective is to describe the clinicopathologic characteristics and treatment outcomes of our patients.
Methods Used Retrospective analysis conducted on patients diagnosed with HLTV1+ ATLL at KCH and UHB between 2005 and 2017. Diagnosis of ATLL was established based on clinical history, pathological findings and HTLV1 serum positivity. IP was based on flow cytometry from peripheral blood and/or immunostaining from lymph node biopsy. Outcomes calculated by log-rank test and survival curves estimated by Kaplan-Meier method.
Summary of Results We identified 63 patients with A and L subtypes with median age 54, female predominance (65%), and 95% Ann Arbor stage III/IV. Organ involvement at presentation as follows: lymphadenopathy (80%), bone marrow (63%), hepatomegaly (25%), skin lesions (24%), bone lesions (23%), splenomegaly (21%), pleural effusions (14%), lung (11%), and CNS (8%). Most patients received upfront EPOCH (78%) or CHOP chemotherapy (14%). The objective response rate (ORR) was 47%, median duration response 2.1 months, and median OS 5.8 months. Median progression-free survival (PFS) was 4 months. Of patients with relapsed/refractory (R/R) disease (85%), 35 patients (66%) received 2nd line treatment (mostly ICE chemotherapy: 27%) with ORR 21%, median OS 2.2 months, and median PFS 2 months. Atypical IP was associated with shorter OS and PFS.
Conclusions Our large cohort of Caribbean patients with acute and lymphomatous subtypes of ATLL suggests a chemo-refractory and aggressive disease, highlighting the need for novel therapies. Incidence of atypical immunophenotype was associated with worse PFS and OS.




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