PRIMARY MEDIASTINAL B‐CELL LYMPHOMA IN CHILDREN, ADOLESCENTS, AND YOUNG ADULTS
نویسندگان
چکیده
Although previously characterized as a subtype of diffuse large B-cell lymphoma, primary mediastinal lymphoma (PMBCL) is now recognized distinct NHL with unique clinical and biologic features, many which overlap classic Hodgkin lymphoma. PMBCL has peak incidence in the adolescent young adult (AYA) population and, unlike other subtypes NHL, higher among females. Recent studies into molecular biology have revealed frequent amplifications 9p24.1 includes locus for PD-L1 genomic alterations CIITA, CD58, B2M, likely further contribute to tumor immune evasion. Treatment varies across centers no single standard care. When treated on historic pediatric protocols designed mature B-NHL, children AYAs inferior outcomes compared patients DLBCL same protocol. trials sought improve addition rituximab modifications regimens and/or adoption dose-adjusted EPOCH-R (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, rituximab) chemotherapy regimen. Despite this, prospective multicenter remain suboptimal. There strong rationale checkpoint blockade including key features outlined above well data from phase II adults showing efficacy pembrolizumab nivolumab relapsed setting. As such, we are conducting III trial evaluating role untreated PMCBL. In this session will review current state science provide an update our ongoing trial. Keywords: extranodal non-Hodgkin (pediatric, adolescent, adult) No conflicts interests pertinent abstract.
منابع مشابه
Hodgkin Lymphoma in children, adolescents and young adults
Englund, A. 2017. Hodgkin Lymphoma in children, adolescents and young adults. Digital Comprehensive Summaries of Uppsala Dissertations from the Faculty of Medicine 1314. 67 pp. Uppsala: Acta Universitatis Upsaliensis. ISBN 978-91-554-9851-1. Hodgkin lymphoma (HL) is a heterogeneous condition varying from engaging one single lymph node site to a widespread condition. The prognosis with contempor...
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ژورنال
عنوان ژورنال: Hematological Oncology
سال: 2023
ISSN: ['1099-1069', '0278-0232']
DOI: https://doi.org/10.1002/hon.3163_29