title: Adult T-cell leukemia/lymphoma in the Caribbean cohort is a distinct clinical entity with dismal response to conventional chemotherapy.
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.
authors.affiliation: Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Monica
authors.initials: M
authors.lastname: Zell
authors.affiliation: Department of Oncology, Montefiore Medical Center/ Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Amer
authors.initials: A
authors.lastname: Assal
authors.affiliation: Department of Oncology, Montefiore Medical Center/ Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Olga
authors.initials: O
authors.lastname: Derman
authors.affiliation: Department of Oncology, Montefiore Medical Center/ Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Noah
authors.initials: N
authors.lastname: Kornblum
authors.affiliation: Department of Oncology, Montefiore Medical Center/ Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Ramakrishna
authors.initials: R
authors.lastname: Battini
authors.affiliation: Department of Pathology, Montefiore Medical Center/ Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Yanhua
authors.initials: Y
authors.lastname: Wang
authors.affiliation: Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Deepa M
authors.initials: DM
authors.lastname: Narasimhulu
authors.affiliation: Department of Oncology, Montefiore Medical Center/ Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Ioannis
authors.initials: I
authors.lastname: Mantzaris
authors.affiliation: Department of Oncology, Montefiore Medical Center/ Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Aditi
authors.initials: A
authors.lastname: Shastri
authors.affiliation: Department of Oncology, Montefiore Medical Center/ Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Amit
authors.initials: A
authors.lastname: Verma
authors.affiliation: Department of Cell Biology, Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Hilda
authors.initials: H
authors.lastname: Ye
authors.affiliation: Department of Oncology, Montefiore Medical Center/ Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Ira
authors.initials: I
authors.lastname: Braunschweig
authors.affiliation: Department of Oncology, Montefiore Medical Center/ Albert Einstein College of Medicine, Bronx, NY, USA.
authors.forename: Murali
authors.initials: M
authors.lastname: Janakiram
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D000998:Antiviral Agents / Q000627:therapeutic use; D017691:Caribbean Region / Q000453:epidemiology; D015331:Cohort Studies; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000453:epidemiology* / Q000473:pathology* / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014184:Transplantation, Homologous; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D000998:Antiviral Agents
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