TY - JOUR
T1 - EBV-positive diffuse large B-cell lymphoma, not otherwise specified
T2 - 2022 update on diagnosis, risk-stratification, and management
AU - Malpica, Luis
AU - Marques-Piubelli, Mario L.
AU - Beltran, Brady E.
AU - Chavez, Julio C.
AU - Miranda, Roberto N.
AU - Castillo, Jorge J.
N1 - Publisher Copyright:
© 2022 Wiley Periodicals LLC.
PY - 2022/7
Y1 - 2022/7
N2 - Disease Overview: Epstein Barr virus-positive (EBV+) diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS) is an entity included in the WHO classification of lymphoid neoplasms since 2016. EBV+ DLBCL, NOS, is an aggressive B-cell lymphoma associated with EBV infection, and a poor prognosis with standard chemotherapeutic approaches. Diagnosis: The diagnosis is made through a careful pathological evaluation. Detection of EBV-encoded RNA (EBER) is considered standard for diagnosis; however, a clear cutoff for percentage of positive cells has not been defined. The differential diagnosis includes plasmablastic lymphoma (PBL), DLBCL associated with chronic inflammation, primary effusion lymphoma (PEL), among others. Risk-Stratification: The International Prognostic Index (IPI) and the Oyama score can be used for risk-stratification. The Oyama score includes age >70 years and presence of B symptoms. The expression of CD30 and PD-1/PD-L1 are emerging as potential adverse but targetable biomarkers. Management: Patients with EBV+ DLBCL, NOS, should be staged and managed following similar guidelines than patients with EBV-negative DLBCL. EBV+ DLBCL, NOS, however, might have a worse prognosis than EBV-negative DLBCL in the era of chemoimmunotherapy. Therefore, the inclusion of patients in clinical trials when available is recommended. There is an opportunity to study and develop targeted therapy in the management of patients with EBV+ DLBCL, NOS.
AB - Disease Overview: Epstein Barr virus-positive (EBV+) diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS) is an entity included in the WHO classification of lymphoid neoplasms since 2016. EBV+ DLBCL, NOS, is an aggressive B-cell lymphoma associated with EBV infection, and a poor prognosis with standard chemotherapeutic approaches. Diagnosis: The diagnosis is made through a careful pathological evaluation. Detection of EBV-encoded RNA (EBER) is considered standard for diagnosis; however, a clear cutoff for percentage of positive cells has not been defined. The differential diagnosis includes plasmablastic lymphoma (PBL), DLBCL associated with chronic inflammation, primary effusion lymphoma (PEL), among others. Risk-Stratification: The International Prognostic Index (IPI) and the Oyama score can be used for risk-stratification. The Oyama score includes age >70 years and presence of B symptoms. The expression of CD30 and PD-1/PD-L1 are emerging as potential adverse but targetable biomarkers. Management: Patients with EBV+ DLBCL, NOS, should be staged and managed following similar guidelines than patients with EBV-negative DLBCL. EBV+ DLBCL, NOS, however, might have a worse prognosis than EBV-negative DLBCL in the era of chemoimmunotherapy. Therefore, the inclusion of patients in clinical trials when available is recommended. There is an opportunity to study and develop targeted therapy in the management of patients with EBV+ DLBCL, NOS.
UR - http://www.scopus.com/inward/record.url?scp=85129867824&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85129867824&partnerID=8YFLogxK
U2 - 10.1002/ajh.26579
DO - 10.1002/ajh.26579
M3 - Article
C2 - 35472248
AN - SCOPUS:85129867824
SN - 0361-8609
VL - 97
SP - 951
EP - 965
JO - American journal of hematology
JF - American journal of hematology
IS - 7
ER -