The molecular pathogenesis of Hodgkin lymphoma.
Histopathology
; 58(1): 15-25, 2011 Jan.
Article
in En
| MEDLINE
| ID: mdl-21261680
ABSTRACT
Hodgkin lymphoma (HL) is an unusual malignancy in that the tumour cells, the Hodgkin and Reed-Sternberg (HRS) cells, are a minor component of the tumour mass, the bulk of which is a mixed cellular infiltrate. There is compelling evidence that HRS cells are clonal B cells that have lost their B cell phenotype. Mature B cells lacking B cell receptors would normally die by apoptosis, and therefore HRS cells must have developed mechanisms to facilitate survival. The escape from apoptosis and transcriptional reprogramming of HRS cells are interlinked and appear central to disease pathogenesis. Epstein-Barr virus (EBV) is present in the HRS cells of a proportion of cases and expresses genes with a plausible oncogenic function. It is likely that EBV plays a role in reprogramming and survival through dysregulation of several signalling networks and transcription factors, including nuclear factor (NF)-κB. Activation of NF-κB is a feature of all HRS cells and gene mutations affecting this pathway appear common in EBV-negative HL. The HRS cell furthers its own survival by attracting a supportive microenvironment of immune and stromal cells, and suppressing local immune responsiveness. Although many questions remain unanswered, the last two decades have witnessed a considerable increase in our knowledge of this complex disease.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Hodgkin Disease
Type of study:
Etiology_studies
Limits:
Animals
/
Humans
Language:
En
Journal:
Histopathology
Year:
2011
Document type:
Article
Affiliation country:
United kingdom