Challenging our understanding of B-cell lymphomagenesis and risk: Paediatric high-grade B-cell lymphoma, not otherwise specified with a DDX3X::MLLT10 fusion and an IGH deletion

Pediatr Blood Cancer. 2024 Mar;71(3):e30810. doi: 10.1002/pbc.30810. Epub 2023 Dec 16.

Abstract

We report a unique case of high-grade B-cell lymphoma, not otherwise specified in a 5-year-old child. Whole-genome sequencing revealed a DDX3X::MLLT10 fusion, usually seen in T-cell acute lymphoblastic leukaemia (ALL). This suggests the novel idea that MLLT10 fusions are capable of driving B-cell malignancies. An IGH deletion usually only seen in adults was also found. These unique genetic findings provide novel insights into B-cell lymphomagenesis. The child remains in remission 7 year post chemotherapy, which demonstrates that novel complex molecular findings do not always denote high-risk disease.

Keywords: HGBL; MLLT10; NOS; lymphoma.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • B-Lymphocytes
  • Child
  • Child, Preschool
  • DEAD-box RNA Helicases / genetics
  • Humans
  • Lymphoma, B-Cell* / genetics
  • Lymphoma, B-Cell* / pathology
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma* / genetics
  • Transcription Factors / genetics

Substances

  • Transcription Factors
  • DDX3X protein, human
  • DEAD-box RNA Helicases
  • MLLT10 protein, human