Molecular insights into pathogenesis and targeted therapy of peripheral T cell lymphoma
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Experimental Hematology & Oncology Open Access
REVIEW
Molecular insights into pathogenesis and targeted therapy of peripheral T cell lymphoma Caiqin Xie1†, Xian Li1†, Hui Zeng2* and Wenbin Qian1,3*
Abstract Peripheral T-cell lymphomas (PTCLs) are biologically and clinically heterogeneous diseases almost all of which are associated with poor outcomes. Recent advances in gene expression profiling that helps in diagnosis and prognostication of different subtypes and next-generation sequencing have given new insights into the pathogenesis and molecular pathway of PTCL. Here, we focus on a broader description of mutational insights into the common subtypes of PTCL including PTCL not other specified type, angioimmunoblastic T-cell lymphoma, anaplastic large cell lymphoma, and extra-nodal NK/T cell lymphoma, nasal type, and also present an overview of new targeted therapies currently in various stages of clinical trials. Keywords: Peripheral T-cell lymphoma, Molecular genetic characteristics, Gene mutation, Targeted therapy Among malignant lymphomas, the peripheral T-cell lymphoma (PTCL) constitutes around 10–15% of all NonHodgkin’s lymphoma (NHL) in western countries, but, in Asian the proportion will increase to 20–30%. Currently, the World Health Organization (WHO) classification recognizes at least 29 subtypes of PTCL. Within this heterogeneous disease group, the most common subtype is PTCL not other specified type (PTCL-NOS), followed by angioimmunoblastic T-cell lymphoma (AITL), anaplastic large cell lymphoma (ALCL), and extra-nodal NK/T cell lymphoma, nasal type (ENKTL) [1]. In general, T-cell lymphomas have worse outcomes as compared to their B-cell counterparts. Most patients did not respond well to anthracycline-based CHOP (cyclophosphamide, *Correspondence: [email protected]; [email protected] † Caiqin Xie and Xian Li—co-first author 1 Department of Hematology, The Second Affiliated Hospital, College of Medicine, Zhejiang University, 88# Jiefang Road, Hangzhou 310009, Zhejiang, People’s Republic of China 2 Department of Hematology, First Affiliated Hospital of Jiaxing University, 1882# Zhonghuan South Road, Jiaxing 314000, People’s Republic of China Full list of author information is available at the end of the article
doxorubicin, vincristine, and prednisone) chemotherapy, with 5-year overall survival (OS) of less than 30%. Patients with refractory recurrent (R/R) PTCL had a worse prognosis [2]. There are challenges in revealing distinct classes of genetic changes that occur in different subtypes of PTCL because relatively low incidence and the complexity of subtype classification. However, in recent years, the widespread application of molecular biology techniques, especially gene expression profiling (GEP) and next-generation sequencing (NGS) technology, has greatly improved our understanding of the genetic changes and pathogenesis of multiple subtypes of PTCL, which were successful in characterizing genetic alterations for AITL, ALCL, adult T-cell leukemia/lymphoma (ATLL), and PTCL-NOS [2]. In this revie
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