TY - JOUR
T1 - Expanding the Spectrum of EBV-positive Marginal Zone Lymphomas
T2 - A Lesion Associated With Diverse Immunodeficiency Settings
AU - Gong, Shunyou
AU - Crane, Genevieve M.
AU - McCall, Chad M.
AU - Xiao, Wenbin
AU - Ganapathi, Karthik A.
AU - Cuka, Nathan
AU - Davies-Hill, Theresa
AU - Xi, Liqiang
AU - Raffeld, Mark
AU - Pittaluga, Stefania
AU - Duffield, Amy S.
AU - Jaffe, Elaine S.
N1 - Funding Information:
Conflicts of Interest and Source of Funding: Supported by the Intramural Research Budget of the Center for Cancer Research, National Cancer Insitute, National Institutes of Health.
PY - 2018/6/27
Y1 - 2018/6/27
N2 - Traditionally low-grade B-cell lymphomas have been excluded from the category of monomorphic posttransplant lymphoproliferative disorders. However, recent reports identified Epstein-Barr virus-positive (EBV+) extranodal marginal zone lymphomas (MZL), almost exclusively seen in the posttransplant setting. Some reported cases responded to reduced immunosuppression, suggesting that they should be considered as a form of posttransplant lymphoproliferative disorders. We identified 10 cases of EBV+ MZL, 9 in extranodal sites and 1 presenting in lymph node. Two cases arose following solid organ transplantation, but other settings included iatrogenic immunosuppression for rheumatoid arthritis (2); prior chemotherapy (2); congenital immune deficiency (1); and increased age (3), as the only potential cause of immune dysfunction. There were 4 males and 6 females; age range 18 to 86. The atypical plasmacytoid and/or monocytoid B cells were positive for EBV in all cases, with either latency I or II in all cases tested. Monotypic light chain expression was shown in all with 6 cases positive for IgG, and 2 for IgM, undetermined in 2. Clonal immunoglobulin gene rearrangement was positive in all cases with successful amplification. MYD88 L265P was wild type in the 6 cases tested. We show that EBV+ MZLs can arise in a variety of clinical settings, and are most often extranodal. Treatment varied, but most patients had clinically indolent disease with response to reduction of immune suppression, or immunochemotherapy.
AB - Traditionally low-grade B-cell lymphomas have been excluded from the category of monomorphic posttransplant lymphoproliferative disorders. However, recent reports identified Epstein-Barr virus-positive (EBV+) extranodal marginal zone lymphomas (MZL), almost exclusively seen in the posttransplant setting. Some reported cases responded to reduced immunosuppression, suggesting that they should be considered as a form of posttransplant lymphoproliferative disorders. We identified 10 cases of EBV+ MZL, 9 in extranodal sites and 1 presenting in lymph node. Two cases arose following solid organ transplantation, but other settings included iatrogenic immunosuppression for rheumatoid arthritis (2); prior chemotherapy (2); congenital immune deficiency (1); and increased age (3), as the only potential cause of immune dysfunction. There were 4 males and 6 females; age range 18 to 86. The atypical plasmacytoid and/or monocytoid B cells were positive for EBV in all cases, with either latency I or II in all cases tested. Monotypic light chain expression was shown in all with 6 cases positive for IgG, and 2 for IgM, undetermined in 2. Clonal immunoglobulin gene rearrangement was positive in all cases with successful amplification. MYD88 L265P was wild type in the 6 cases tested. We show that EBV+ MZLs can arise in a variety of clinical settings, and are most often extranodal. Treatment varied, but most patients had clinically indolent disease with response to reduction of immune suppression, or immunochemotherapy.
KW - Epstein-Barr virus
KW - immunodeficiency
KW - immunosenescence
KW - immunosuppression
KW - MALT lymphoma
KW - marginal zone lymphoma
KW - posttransplant lymphoproliferative disease
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U2 - 10.1097/PAS.0000000000001113
DO - 10.1097/PAS.0000000000001113
M3 - Article
C2 - 29957733
AN - SCOPUS:85049218399
VL - 42
SP - 1306
EP - 1316
JO - American Journal of Surgical Pathology
JF - American Journal of Surgical Pathology
SN - 0147-5185
IS - 10
ER -