In:
Hematology, American Society of Hematology, Vol. 2009, No. 1 ( 2009-01-01), p. 491-496
Kurzfassung:
Hodgkin lymphoma (HL) is derived from mature B cells and subdivided into classical HL and nodular lymphocyte predominant HL (NLPHL). HL is unique among human B cell lymphomas because of the rarity of the lymphoma cells, the Hodgkin and Reed-Sternberg (HRS) cells in classical HL and the lymphocyte-predominant (LP) cells in NLPHL, which usually account for 0.1% to 10% of the cells in the affected tissues. Moreover, HRS cells are unique in the extent to which they have lost their B cell–typical gene expression pattern. Deregulation of transcription factor networks plays a key role in this reprogramming process. HRS cells show strong constitutive activity of the transcription factor NF-κB. Multiple mechanisms likely contribute to this deregulated activation, including signaling through particular receptors and genetic lesions. Inactivating mutations in the TNFAIP3 tumor suppressor gene, encoding a negative regulator of NF-κB activity, were recently identified in about 40% of patients with classical HL. HRS cells are latently infected by Epstein-Barr virus in about 40% of patients, and an important role of this virus in HL pathogenesis—in particular for cases in which HRS cells had lost the capacity to express a B-cell receptor due to destructive somatic mutation—was recently substantiated.
Materialart:
Online-Ressource
ISSN:
1520-4391
,
1520-4383
DOI:
10.1182/asheducation-2009.1.491
Sprache:
Englisch
Verlag:
American Society of Hematology
Publikationsdatum:
2009
ZDB Id:
2084287-9
