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

Br J Haematol. 2021 Jan;192(2):354-365. doi: 10.1111/bjh.17043. Epub 2020 Sep 3.

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.

Keywords: B-cell lymphoma; Burkitt lymphoma; murine cancer models; patient derived xenograft; relapse.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Burkitt Lymphoma / genetics
  • Burkitt Lymphoma / pathology*
  • Burkitt Lymphoma / therapy
  • Child
  • Disease Models, Animal
  • Female
  • Gene Expression Regulation, Neoplastic
  • Heterografts / pathology
  • Humans
  • Male
  • Mice
  • Neoplasm Transplantation
  • Tumor Cells, Cultured