Mantle cell lymphoma - advances in molecular biology, prognostication and treatment approaches

Br J Haematol. 2021 Oct;195(2):162-173. doi: 10.1111/bjh.17419. Epub 2021 Mar 30.

Abstract

Mantle cell lymphoma (MCL) is clinically characterised by its heterogenous behaviour with courses ranging from indolent cases that do not require therapy for years to highly aggressive MCL with a very limited prognosis. A better understanding of the complex biology of MCL has already led to the approval of several innovative agents, expanding the landscape of MCL therapies and improving therapeutic options especially for refractory/relapsed (R/R) disease. Nevertheless, to further optimise MCL treatment, early identification of individual risk profile and risk-adapted, patient-tailored choice of therapeutic strategy needs to be prospectively incorporated into clinical patient management. The present review highlights recent advances in deciphering the molecular background of MCL, the definition of prognostically relevant factors and the identification of potential druggable targets and summarises current treatment recommendations for primary and R/R MCL including novel targeted therapies.

Keywords: Mantle cell lymphoma; genetics; pathogenesis; prognostication; therapy.

Publication types

  • Review

MeSH terms

  • Aged
  • Female
  • High-Throughput Nucleotide Sequencing / methods
  • Humans
  • Immunoglobulin Heavy Chains / genetics
  • Lymphoma, Mantle-Cell / diagnosis
  • Lymphoma, Mantle-Cell / genetics*
  • Lymphoma, Mantle-Cell / metabolism
  • Lymphoma, Mantle-Cell / pathology*
  • Male
  • Molecular Biology / methods*
  • Molecular Biology / statistics & numerical data
  • Molecular Targeted Therapy / methods*
  • Neoplasm Staging / methods
  • Prognosis
  • Risk Assessment
  • SOXC Transcription Factors
  • Severity of Illness Index
  • Survival Rate / trends

Substances

  • Immunoglobulin Heavy Chains
  • SOX11 protein, human
  • SOXC Transcription Factors