Primary central nervous system lymphoma: status and advances in diagnosis, molecular pathogenesis, and treatment
Abstract. Primary central nervous system lymphoma (PCNSL) is a rare group of extra-nodal non-Hodgkin lymphoma which is confined to the central nervous system or eyes. This article aims to present a brief profile of PCNSL diagnosis and treatment in immunocompetent patients. The authors retrieved info...
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doaj-8ce74e0d61174bda9098128571754a672020-12-02T07:58:52ZengWolters KluwerChinese Medical Journal0366-69992542-56412020-06-01133121462146910.1097/CM9.0000000000000844202006200-00013Primary central nervous system lymphoma: status and advances in diagnosis, molecular pathogenesis, and treatmentYan Zhang0Dao-Bin Zhou1Peng Lyu2Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.Abstract. Primary central nervous system lymphoma (PCNSL) is a rare group of extra-nodal non-Hodgkin lymphoma which is confined to the central nervous system or eyes. This article aims to present a brief profile of PCNSL diagnosis and treatment in immunocompetent patients. The authors retrieved information from the PubMed database up to September 2019. The annual incidence of PCNSL increased over the last four decades. The prognosis of PCNSL has improved mainly due to the introduction and wide-spread use of high-dose methotrexate, which is now the backbone of all first-line treatment polychemotherapy regimens. Gene expression profiling and next-generation sequencing analyses have revealed mutations that induce activation of nuclear factor-κB, B cell antigen receptor, and Janus kinases/signal transducer and activator of transcription proteins signal pathways. Some novel agents are investigated in the treatment of relapsed PCNSL including immunotherapy and targeted therapy. In particular, lenalidomide and ibrutinib have demonstrated durable efficiency. Treatment of PCNSL has evolved in the last 40 years and survival outcomes have improved in most patient groups, but there is still room to improve outcome by optimizing current chemotherapy and novel agents.http://journals.lww.com/10.1097/CM9.0000000000000844 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Yan Zhang Dao-Bin Zhou Peng Lyu |
spellingShingle |
Yan Zhang Dao-Bin Zhou Peng Lyu Primary central nervous system lymphoma: status and advances in diagnosis, molecular pathogenesis, and treatment Chinese Medical Journal |
author_facet |
Yan Zhang Dao-Bin Zhou Peng Lyu |
author_sort |
Yan Zhang |
title |
Primary central nervous system lymphoma: status and advances in diagnosis, molecular pathogenesis, and treatment |
title_short |
Primary central nervous system lymphoma: status and advances in diagnosis, molecular pathogenesis, and treatment |
title_full |
Primary central nervous system lymphoma: status and advances in diagnosis, molecular pathogenesis, and treatment |
title_fullStr |
Primary central nervous system lymphoma: status and advances in diagnosis, molecular pathogenesis, and treatment |
title_full_unstemmed |
Primary central nervous system lymphoma: status and advances in diagnosis, molecular pathogenesis, and treatment |
title_sort |
primary central nervous system lymphoma: status and advances in diagnosis, molecular pathogenesis, and treatment |
publisher |
Wolters Kluwer |
series |
Chinese Medical Journal |
issn |
0366-6999 2542-5641 |
publishDate |
2020-06-01 |
description |
Abstract. Primary central nervous system lymphoma (PCNSL) is a rare group of extra-nodal non-Hodgkin lymphoma which is confined to the central nervous system or eyes. This article aims to present a brief profile of PCNSL diagnosis and treatment in immunocompetent patients. The authors retrieved information from the PubMed database up to September 2019. The annual incidence of PCNSL increased over the last four decades. The prognosis of PCNSL has improved mainly due to the introduction and wide-spread use of high-dose methotrexate, which is now the backbone of all first-line treatment polychemotherapy regimens. Gene expression profiling and next-generation sequencing analyses have revealed mutations that induce activation of nuclear factor-κB, B cell antigen receptor, and Janus kinases/signal transducer and activator of transcription proteins signal pathways. Some novel agents are investigated in the treatment of relapsed PCNSL including immunotherapy and targeted therapy. In particular, lenalidomide and ibrutinib have demonstrated durable efficiency. Treatment of PCNSL has evolved in the last 40 years and survival outcomes have improved in most patient groups, but there is still room to improve outcome by optimizing current chemotherapy and novel agents. |
url |
http://journals.lww.com/10.1097/CM9.0000000000000844 |
work_keys_str_mv |
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