Hodgkin lymphoma

J Clin Invest. 2012 Oct;122(10):3439-47. doi: 10.1172/JCI61245. Epub 2012 Oct 1.

Abstract

Hodgkin lymphoma (HL), a B cell-derived cancer, is one of the most common lymphomas. In HL, the tumor cells--Hodgkin and Reed-Sternberg (HRS) cells--are usually very rare in the tissue. Although HRS cells are derived from mature B cells, they have largely lost their B cell phenotype and show a very unusual co-expression of markers of various hematopoietic cell types. HRS cells show deregulated activation of multiple signaling pathways and transcription factors. The activation of these pathways and factors is partly mediated through interactions of HRS cells with various other types of cells in the microenvironment, but also through genetic lesions. The transforming events involved in the pathogenesis of HL are only partly understood, but mutations affecting the NF-κB and JAK/STAT pathways are frequent. The dependency of HRS cells on microenvironmental interactions and deregulated signaling pathways may offer novel strategies for targeted therapies.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Antigens, Differentiation, B-Lymphocyte / analysis
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • B-Lymphocytes / pathology
  • Cell Lineage
  • Cell Transformation, Neoplastic / genetics
  • Chemoradiotherapy
  • Clinical Trials as Topic
  • Epstein-Barr Virus Infections / pathology
  • Gene Expression Regulation, Neoplastic
  • Germ-Line Mutation
  • Herpesvirus 4, Human / genetics
  • Herpesvirus 4, Human / pathogenicity
  • Hodgkin Disease* / classification
  • Hodgkin Disease* / etiology
  • Hodgkin Disease* / genetics
  • Hodgkin Disease* / pathology
  • Hodgkin Disease* / therapy
  • Hodgkin Disease* / virology
  • Humans
  • Molecular Targeted Therapy
  • NF-kappa B / metabolism
  • Neoplasm Proteins / biosynthesis
  • Neoplasm Proteins / genetics
  • Neoplasm Proteins / physiology
  • Phenotype
  • Reed-Sternberg Cells / immunology
  • Reed-Sternberg Cells / pathology
  • Signal Transduction / genetics
  • Signal Transduction / physiology
  • Transcription Factors / genetics
  • Transcription Factors / physiology
  • Tumor Microenvironment
  • Tumor Virus Infections / pathology

Substances

  • Antigens, Differentiation, B-Lymphocyte
  • NF-kappa B
  • Neoplasm Proteins
  • Transcription Factors