Elsevier

Experimental Hematology

Volume 51, July 2017, Pages 7-16
Experimental Hematology

Review
Adult T-type lymphoblastic lymphoma: Treatment advances and prognostic indicators

https://doi.org/10.1016/j.exphem.2017.04.002Get rights and content
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Highlights

  • T-lymphoblastic lymphoma is a rare, aggressive neoplasm of precursor T cells.

  • Survival in adults is poor with use of non-Hodgkin's lymphoma-like regimens.

  • Survival has improved with use of pediatric lymphoblastic leukemia-like regimens.

  • Oncogenetic factors can be used to stratify risk in adult patients.

T-cell lymphoblastic lymphoma (T-LBL) is a rare, aggressive neoplasm of precursor T cells that occurs mostly in adolescents and young adults. In this review, we describe the treatment of adult T-LBL with a focus on recent advances using pediatric-inspired acute lymphoblastic leukemia regimens, which have greatly improved outcome. We also discuss the development of prognostic indicators for T-LBL, especially oncogenetic factors, that can identify patients at higher risk of relapse and may help further extend T-LBL patient survival. Pediatric-inspired acute lymphoblastic leukemia regimens have the potential to become the treatment of choice for adult T-LBL, and they might also reduce the need for other longstanding T-LBL interventions, particularly mediastinal irradiation and stem cell transplantation.

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