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

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

Research output: Contribution to journalArticle

18 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)51981-51990
Number of pages10
JournalOncotarget
Volume7
Issue number32
DOIs
StatePublished - Aug 1 2016

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Keywords

  • Adult T-cell leukemia/lymphoma (ATLL)
  • Allogeneic stem cell transplantation
  • Human T-cell lymphotropic virus type-1 (HTLV-1)
  • Non-Hodgkin lymphoma
  • T-cell lymphoma

ASJC Scopus subject areas

  • Oncology

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