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

Abstract

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.

Radiation Therapy in Precursor T-Lymphoblastic Lymphoma/Leukemia

“…T-cell lymphoblastic lymphoma (T-LBL) is a neoplasm of precursor T-cells of lymphoblast origin. T-LBL was first described in 1916 and T-LBL in adults represents only 1À2% of all non-Hodgkin lymphomas (NHLs) (Lepretre et al 2017). In contrast, T-LBL constitutes about 25% of all adult ALL (Hoelzer and Gokbuget 2009).…”

“…Cytogenetic abnormalities and clonal rearrangements of the T-cell receptor (TCR) are frequently found in T-LBL and may be involved in its pathogenesis by driving altered expression of T-cell oncogenes (Lepretre et al 2017). The most common cytogenetic abnormalities in T-LBL involve the 14q11-13 region, the site of the TCR-a, and TCR-d genes.…”

“…Pediatric-inspirited ALL treatment regimes, e.g., BFM-based protocol of the GMALL study group (Gökbuget et al 2014) or the treatment protocol of the GRAALL (Lepretre et al 2016), consist of an intensive multidrug chemotherapy induction phase (often incorporating an anthracycline, cyclophosphamide, vincristine, asparaginase, and a steroid) and multiple courses of high-dose consolidation treatments with CNS prophylaxis, lasting up to 1 year, followed sometimes by maintenance chemotherapy for as many as 2 years. Adult LBL-patients treated with such protocols have shown significantly improved complete remission rates of 76-93% and have prolonged event-/progression-free survival of 62-76% after 3-8 years and overall survival of up to 65-69% after 3-5 years (Lepretre et al 2017).…”

Antitumor effect of triptolide in T-cell lymphoblastic lymphoma by inhibiting cell viability, invasion, and epithelial–mesenchymal transition via regulating the PI3K/AKT/mTOR pathway

“…However, 5-year overall survival is still poor for T-LBL mainly due to relapse, often as a result of spread to CNS. 4 Thus, suppressing the viability and invasion ability of T-LBL tumor cells can ameliorate T-LBL and improve prognosis of patients.…”

T‐cell lymphomas associated gene expression signature: Bioinformatics analysis based on gene expression Omnibus

“…T‐cell neoplasms originate from precursor T cells or mature T cells at different courses of differentiation with diverse morphological patterns, cell phenotypes, genetic criteria, and clinical characters. T‐cell lymphoblastic lymphoma (T‐LBL) and T‐cell acute lymphoblastic leukemia (T‐ALL) are neoplasms that originate from T‐cell precursors . T‐cell prolymphocytic leukemia (T‐PLL), peripheral T‐cell lymphoma, NOS (PTCL‐NOS), ALK‐positive anaplastic large‐cell lymphoma (ALCL), ALK‐negative ALCL, and angioimmunoblastic T‐cell lymphoma (AITL), etc.…”