Biology and therapy of primary mediastinal B-cell lymphoma: current status and future directions.
Adult
Antigens, Neoplasm
/ immunology
Clonal Anergy
/ genetics
Female
Humans
Immunotherapy
Janus Kinases
/ metabolism
Lymphoma, B-Cell
/ diagnosis
Male
Mediastinal Neoplasms
/ diagnosis
Middle Aged
NF-kappa B
/ metabolism
STAT Transcription Factors
/ metabolism
Signal Transduction
T-Lymphocytes
/ immunology
Tumor Microenvironment
/ genetics
Young Adult
haematological oncology
malignant lymphomas
non-Hodgkin lymphoma
tumour biology
tumour immunotherapy
Journal
British journal of haematology
ISSN: 1365-2141
Titre abrégé: Br J Haematol
Pays: England
ID NLM: 0372544
Informations de publication
Date de publication:
04 2019
04 2019
Historique:
pubmed:
12
2
2019
medline:
20
2
2020
entrez:
12
2
2019
Statut:
ppublish
Résumé
Primary mediastinal B-cell lymphoma (PMBCL) is a distinct disease closely related to classical nodular sclerosing Hodgkin lymphoma. Conventional diagnostic paradigms utilising clinical, morphological and immunophenotypical features can be challenging due to overlapping features with other B-cell lymphomas. Reliable diagnostic and prognostic biomarkers that are applicable to the conventional diagnostic laboratory are largely lacking. Nuclear factor kappa B (NF-κB) and Janus kinase/signal transducers and activators of transcription (JAK-STAT) signalling pathways are characteristically dysregulated in PMBCL and implicated in several aspects of disease pathogenesis, and the latter pathway in host immune evasion. The tumour microenvironment is manipulated by PMBCL tumours to avoid T-cell mediated destruction via strategies that include loss of tumour cell antigenicity, T-cell exhaustion and activation of suppressive T-regulatory cells. R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone) and DA-EPOCH-R (dose-adjusted etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin, rituximab) are the most common first-line immunochemotherapy regimens. End of treatment positron emission tomography scans are the recommended imaging modality and are being evaluated to stratify patients for radiotherapy. Relapsed/refractory disease has a relatively poor outcome despite salvage immunochemotherapy and subsequent autologous stem cell transplantation. Novel therapies are therefore being developed for treatment-resistant disease, targeting aberrant cellular signalling and immune evasion.
Identifiants
pubmed: 30740662
doi: 10.1111/bjh.15778
pmc: PMC6594147
doi:
Substances chimiques
Antigens, Neoplasm
0
NF-kappa B
0
STAT Transcription Factors
0
Janus Kinases
EC 2.7.10.2
Banques de données
GENBANK
['NCT03346642', 'NCT00078949', 'NCT02568553', 'NCT03038672', 'NCT02950220', 'NCT03484819', 'NCT02576990', 'NCT02631044', 'NCT03601819', 'NCT02747732', 'NCT01599559', 'NCT03625037', 'NCT02348216']
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
25-41Informations de copyright
© 2019 The Authors British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.
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