Adult T-cell leukemia/lymphoma in the Caribbean cohort is a distinct clinical entity with dismal response to conventional chemotherapy
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Monica Zell1,*, Amer Assal2,*, Olga Derman2, Noah Kornblum2, Ramakrishna Battini2, Yanhua Wang3, Deepa M. Narasimhulu1, Ioannis Mantzaris2, Aditi Shastri2, Amit Verma2, Hilda Ye4, Ira Braunschweig2, Murali Janakiram2
1Albert Einstein College of Medicine, Bronx, NY, USA
2Department of Oncology, Montefiore Medical Center/ Albert Einstein College of Medicine, Bronx, NY, USA
3Department of Pathology, Montefiore Medical Center/ Albert Einstein College of Medicine, Bronx, NY, USA
4Department of Cell Biology, Albert Einstein College of Medicine, Bronx, NY, USA
*These authors have contributed equally to this work
Murali Janakiram, email: [email protected]
Keywords: human T-cell lymphotropic virus type-1 (HTLV-1), adult T-cell leukemia/lymphoma (ATLL), non-Hodgkin lymphoma, T-cell lymphoma, allogeneic stem cell transplantation
Received: January 27, 2016 Accepted: May 09, 2016 Published: June 22, 2016
Adult T-cell leukemia/lymphoma (ATLL) is a rare and aggressive disease caused by human T-cell lymphotropic virus type 1 that predominantly affects Japanese and Caribbean populations. Most studies have focused on Japanese cohorts. We conducted a retrospective analysis of 53 cases of ATLL who presented to our institution between 2003-2014. ATLL in the Caribbean population presents more often as the acute and lymphomatous subtypes, is associated with complex cytogenetics, and has a high rate of CNS involvement. The overall response rate to first-line therapies with anthracycline-based regimens was poor (32%), with a median survival of only 6.9 months. A complete or partial response to first-line regimens was associated with better survival. There was no difference in survival between patients who received chemotherapy alone versus chemotherapy with antiviral agents. Allogeneic transplantation was performed in five patients, two of whom achieved complete remission despite residual or refractory disease. Recipients of allogeneic transplantation had significantly improved overall survival compared to non-transplanted patients. This is the first analysis to describe ATLL pathological features, cytogenetics, and response to standard therapy and transplantation in the Caribbean cohort.
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