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Molecular biology of Hodgkin lymphoma.
| Content Provider | Semantic Scholar |
|---|---|
| Author | Küppers, Ralf |
| Copyright Year | 2009 |
| Abstract | 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-kappaB. 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-kappaB 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. |
| Starting Page | 428 |
| Ending Page | 437 |
| Page Count | 10 |
| File Format | PDF HTM / HTML |
| Alternate Webpage(s) | http://asheducationbook.hematologylibrary.org/content/2009/1/491.full.pdf |
| PubMed reference number | 20008234v1 |
| Alternate Webpage(s) | https://doi.org/10.1182/asheducation-2009.1.491 |
| DOI | 10.1182/asheducation-2009.1.491 |
| Journal | Hematology. American Society of Hematology. Education Program |
| Language | English |
| Access Restriction | Open |
| Subject Keyword | B-Cell Lymphomas B-Lymphocytes Body tissue Chronic Lymphocytic Leukemia Deregulation Diploid Cell Epstein-Barr Virus Gene Expression Leukemia, B-Cell Lymphoma Cell Count Lymphoma, Non-Hodgkin Molecular Diagnostic Techniques NF-kappa B Nodular Lymphocyte Predominant Hodgkin Lymphoma Patients Reprogram Signal Transduction Somatic mutation TRANSCRIPTION FACTOR Tumor Suppressor Genes childhood lymphocyte depletion Hodgkin's lymphoma |
| Content Type | Text |
| Resource Type | Article |
