EBV-positive diffuse large B-cell lymphoma of the elderly: 2016 update on diagnosis, risk-stratification, and management.
Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (DLBCL) of the elderly is a provisional entity included in the 2008 WHO classification of lymphoid neoplasms. It is a disease typically seen in the elderly and thought to be associated with chronic EBV infection and severe immunosuppression with a component of immunosenescence. Recent research, however, has suggested that EBV-positive DLBCL can be seen in younger, immunocompetent patients. The diagnosis of EBV-positive DLBCL of the elderly is made through a careful pathological evaluation. The differential diagnosis includes infectious mononucleosis (specifically in younger patients), lymphomatoid granulomatosis, Hodgkin lymphoma, and gray zone lymphoma, among others. Detection of EBV-encoded RNA (EBER) is considered standard for diagnosis; however, a clear cutoff for positivity has not been defined. 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 is emerging as a potential adverse, and targetable, prognostic factor. Patients with EBV-positive DLBCL should be staged and managed following similar guidelines than patients with EBV-negative DLBCL. It has been suggested, however, that EBV-positive patients have a worse prognosis than EBV-negative counterparts in the era of chemoimmunotherapy. There is an opportunity to study and develop targeted therapy in the management of patients with EBV-positive DLBCL.
Division of Hematologic Malignancies, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts.
Jorge J
JJ
Castillo
Department of Oncology and Radiotherapy, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru.
Brady E
BE
Beltran
Department of Hematopathology, the University of Texas MD Anderson Cancer Center, Houston, Texas.
Roberto N
RN
Miranda
Department of Hematopathology, the University of Texas MD Anderson Cancer Center, Houston, Texas.
Ken H
KH
Young
Division of Malignant Hematology, Moffitt Cancer Center & Research Institute, University of South Florida, Tampa, Florida.
Julio C
JC
Chavez
Department of Hematology and Oncology, George Washington Cancer Center, George Washington University, Washington, DC.
Eduardo M
EM
Sotomayor
D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D000818:Animals; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D000998:Antiviral Agents / Q000627:therapeutic use; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D019468:Disease Management; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D004854:Herpesvirus 4, Human / Q000187:drug effects / Q000502:physiology; D006801:Humans; D016219:Immunotherapy, Adoptive; D007244:Infectious Mononucleosis / Q000175:diagnosis; D008223:Lymphoma / Q000175:diagnosis; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy / Q000821:virology*; D051379:Mice; D008875:Middle Aged; D058990:Molecular Targeted Therapy; D011379:Prognosis; D018570:Risk Assessment; D000069283:Rituximab / Q000008:administration & dosage; D014775:Virus Activation
D000998:Antiviral Agents; D000069283:Rituximab