Lymphoid enhancer binding factor 1 (LEF1) expression is significantly higher in Hodgkin lymphoma associated with Richter syndrome relative to de novo classic Hodgkin lymphoma

• Published in: Annals of diagnostic pathology Vol. 49; p. 151636
• Main Authors: Lyapichev, Kirill A., Sakhdari, Ali, Khoury, Joseph D., O'Malley, Dennis P., El Hussein, Siba, Yin, Cameron C., Patel, Keyur P., Thakral, Beenu, Young, Ken H., Medeiros, L. Jeffrey, Konoplev, Sergej
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.12.2020
• Subjects: Classic Hodgkin lymphoma; EBV; LEF1; Richter syndrome; EBV; Richter syndrome; LEF1; Classic Hodgkin lymphoma
• ISSN: 1092-9134; 1532-8198
• DOI: 10.1016/j.anndiagpath.2020.151636

Lymphoid enhancer binding factor 1 (LEF1) is consistently upregulated in chronic lymphocytic leukemia (CLL) and in a subset of large B cell lymphoma. Knowledge of LEF1 expression in Hodgkin lymphoma is limited. In this study, we used immunohistochemistry to survey LEF1 expression in various subsets of Hodgkin lymphoma, de novo classic Hodgkin lymphoma (CHL) (n = 43), Hodgkin lymphoma associated with Richter syndrome (HL-RS) (n = 20), and nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) (n = 9). LEF1 expression was significantly higher in HL-RS compared with de novo CHL (12/20, 60% vs. 12/43, 28%; p = 0.0248). Only a single case (1/9; 11%) of NLPHL showed LEF1 expression. Epstein-Barr virus encoded RNA (EBER) was detected in 17 (40%) cases of de novo CHL and 14 (70%) HL-RS. Notably, we identified a correlation between LEF1 expression and EBER positivity (p = 0.0488). We concluded that LEF1 is commonly positive in CHL but not in NLPHL, and such a distinction may be helpful in this differential diagnosis. The higher frequency of LEF1 upregulation in HL-RS relative to de novo CHL suggests that these neoplasms might have different underlying pathogenic mechanisms and warrants further investigation. •Lymphoid enhancer binding factor 1 (LEF1) is commonly expressed classic Hodgkin lymphoma (CHL).•LEF1 expression was higher in Hodgkin lymphoma associated with Richter syndrome compared with de novo CHL (60% vs. 28%; p=0.0248).•We identified a correlation between LEF1 expression and EBER positivity (p=0.0488).•Only 11% patients with nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) showed LEF1 expression.•A difference in LEF1 expression in CHL and NLPHL may be helpful in the differential diagnosis.