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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MSK Authors
  1. Julie T Feldstein
    292 Feldstein
  2. Olga L. Bohn
    10 Bohn