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Conventional diagnostic paradigms utilising clinical, morphological and immunophenotypical features can be challenging due to overlapping features with other B\u2010cell lymphomas. Reliable diagnostic and prognostic biomarkers that are applicable to the conventional diagnostic laboratory are largely lacking. Nuclear factor kappa B (<jats:styled-content style=\"fixed-case\">NF<\/jats:styled-content>\u2010\u03baB) and Janus kinase\/signal transducers and activators of transcription (<jats:styled-content style=\"fixed-case\">JAK<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">STAT<\/jats:styled-content>) signalling pathways are characteristically dysregulated in <jats:styled-content style=\"fixed-case\">PMBCL<\/jats:styled-content> and implicated in several aspects of disease pathogenesis, and the latter pathway in host immune evasion. The tumour microenvironment is manipulated by <jats:styled-content style=\"fixed-case\">PMBCL<\/jats:styled-content> tumours to avoid T\u2010cell mediated destruction via strategies that include loss of tumour cell antigenicity, T\u2010cell exhaustion and activation of suppressive T\u2010regulatory cells. R\u2010<jats:styled-content style=\"fixed-case\">CHOP<\/jats:styled-content> (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone) and <jats:styled-content style=\"fixed-case\">DA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">EPOCH<\/jats:styled-content>\u2010R (dose\u2010adjusted etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin, rituximab) are the most common first\u2010line 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\u2010resistant disease, targeting aberrant cellular signalling and immune evasion.<\/jats:p>","DOI":"10.1111\/bjh.15778","type":"journal-article","created":{"date-parts":[[2019,2,11]],"date-time":"2019-02-11T01:54:01Z","timestamp":1549850041000},"page":"25-41","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":69,"title":["Biology and therapy of primary mediastinal B\u2010cell lymphoma: current status and future directions"],"prefix":"10.1111","volume":"185","author":[{"given":"Charlotte","family":"Lees","sequence":"first","affiliation":[{"name":"Blood Cancer Research Group Mater Research University of Queensland Translational Research Institute Brisbane QLD Australia"},{"name":"Princess Alexandra Hospital Southside Clinical Unit Faculty of Medicine University of Queensland Translational Research Institute Brisbane QLD 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