Utility of LRF/pokemon and NOTCH1 protein expression in the distinction between nodular lymphocyte-predominant hodgkin lymphoma and classical hodgkin lymphoma Journal Article
| Authors: | Bohn, O.; Maeda, T.; Filatov, A.; Lunardi, A.; Pandolfi, P. P.; Teruya-Feldstein, J. |
| Article Title: | Utility of LRF/pokemon and NOTCH1 protein expression in the distinction between nodular lymphocyte-predominant hodgkin lymphoma and classical hodgkin lymphoma |
| Abstract: | Classical Hodgkin lymphoma (CHL) and nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) are considered separate entities with different prognosis and treatment. However, morphologic features can be similar and immunohistochemical studies are essential in the distinction; thus, determination of additional biomarkers is of utmost importance. LRF/Pokemon is a proto-oncogene, an interacting partner co-expressed with BCL6 in germinal centers and highly expressed in diffuse large B-cell lymphoma and follicular lymphoma. Conversely, loss of the LRF gene in mouse hematopoietic stem cells results in complete block of early B cell development with concomitant Notch de-repression, indicating its critical role in B versus T cell fate decision at the hematopoietic stem cell stage. For the first time, we show that LRF/Pokemon is predominantly expressed in NLPHL cases as is BCL6 with low to absent NOTCH1 protein expression; while Hodgkin Reed-Sternberg (HRS) cells in CHL show low to absent BCL6 and LRF/Pokemon expression with higher NOTCH1 expression. We illustrate a potential functional interaction between LRF and BCL6 in NLPHL pathogenesis, and differential expression of LRF/Pokemon and NOTCH1 proteins in CHL thus showing differential expression, making for an additional diagnostic marker and therapeutic target. © The Author(s) 2013. |
| Keywords: | hodgkin lymphoma; pokemon; classical hodgkin lymphoma (chl); lrf; nodular lymphocyte-predominant hodgkin lymphoma (nlphl) |
| Journal Title: | International Journal of Surgical Pathology |
| Volume: | 22 |
| Issue: | 1 |
| ISSN: | 1066-8969 |
| Publisher: | Sage Publications |
| Date Published: | 2014-01-01 |
| Start Page: | 6 |
| End Page: | 11 |
| Language: | English |
| DOI: | 10.1177/1066896913513833 |
| PROVIDER: | scopus |
| PMCID: | PMC3971474 |
| PUBMED: | 24326827 |
| DOI/URL: | |
| Notes: | Export Date: 3 March 2014 -- CODEN: IJSPF -- Source: Scopus |
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