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Paediatric Burkitt lymphoma patient-derived xenografts capture disease characteristics over time and are a model for therapy.

Accepted version
Peer-reviewed

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Type

Article

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Authors

Forde, Sorcha 
Matthews, Jamie D 
Jahangiri, Leila 
Lee, Liam C 
Prokoph, Nina 

Abstract

Burkitt lymphoma (BL) accounts for almost two-thirds of all B-cell non-Hodgkin lymphoma (B-NHL) in children and adolescents and is characterised by a MYC translocation and rapid cell turnover. Intensive chemotherapeutic regimens have been developed in recent decades, including the lymphomes malins B (LMB) protocol, which have resulted in a survival rate in excess of 90%. Recent clinical trials have focused on immunochemotherapy, with the addition of rituximab to chemotherapeutic backbones, showing encouraging results. Despite these advances, relapse and refractory disease occurs in up to 10% of patients and salvage options for these carry a dismal prognosis. Efforts to better understand the molecular and functional characteristics driving relapse and refractory disease may help improve this prognosis. This study has established a paediatric BL patient-derived xenograft (PDX) resource which captures and maintains tumour heterogeneity, may be used to better characterise tumours and identify cell populations responsible for therapy resistance.

Description

Keywords

B-cell lymphoma, Burkitt lymphoma, murine cancer models, patient derived xenograft, relapse, Animals, Burkitt Lymphoma, Child, Disease Models, Animal, Female, Gene Expression Regulation, Neoplastic, Heterografts, Humans, Male, Mice, Neoplasm Transplantation, Tumor Cells, Cultured

Journal Title

Br J Haematol

Conference Name

Journal ISSN

0007-1048
1365-2141

Volume Title

192

Publisher

Wiley

Rights

All rights reserved
Sponsorship
Alex Hulme Foundation (unknown)
Alex Hulme Foundation (unknown)
Alex Hulme Foundation