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. 2016 Aug 9;7(32):51981-51990.
doi: 10.18632/oncotarget.10223.

Adult T-cell leukemia/lymphoma in the Caribbean cohort is a distinct clinical entity with dismal response to conventional chemotherapy

Monica Zell  1 , Amer Assal  2 , Olga Derman  2 , Noah Kornblum  2 , Ramakrishna Battini  2 , Yanhua Wang  3 , Deepa M Narasimhulu  1 , Ioannis Mantzaris  2 , Aditi Shastri  2 , Amit Verma  2 , Hilda Ye  4 , Ira Braunschweig  2 , Murali Janakiram  2
Affiliations

Adult T-cell leukemia/lymphoma in the Caribbean cohort is a distinct clinical entity with dismal response to conventional chemotherapy

Monica Zell et al. Oncotarget. .

Abstract

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.

Keywords: T-cell lymphoma; adult T-cell leukemia/lymphoma (ATLL); allogeneic stem cell transplantation; human T-cell lymphotropic virus type-1 (HTLV-1); non-Hodgkin lymphoma.

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Conflict of interest statement

None of the authors have any conflict of interest to disclose.

Figures

Figure 1
Figure 1. Survival in the ATLL Caribbean cohort
A. Overall survival by ATLL subtype B. Overall survival comparing first-line chemotherapy with antiviral agent versus first-line chemotherapy alone C. Overall survival response to first-line treatment D. Overall survival of patients with allogeneic hematopoietic stem transplant compared to overall cohort. HSCT: hematopoietic stem cell transplant; allo HSCT: allogeneic hematopoietic stem cell transplant.
See this image and copyright information in PMC

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