Epstein-Barr virus-positive nodular lymphocyte predominant Hodgkin lymphoma.
Hodgkin lymphoma (HL) is classified into 2 largely distinct subgroups, namely nodular lymphocyte predominant HL (NLPHL) and classic HL (CHL). CHL is further divided into nodular sclerosis, lymphocyte-rich, mixed cellularity (MCCHL) and lymphocyte-depleted (LDCHL) subtypes. In industrialized nations, Epstein-Barr virus (EBV) has been associated with all types of CHL, especially the MCCHL and LDCHL subtypes, but is rare in NLPHL. We report 8 cases of EBV-positive NLPHL occurring in patients in the United States. All 8 patients have no history of immunosuppression and presented with localized or systemic lymphadenopathy. Histologically, 6 cases had a vaguely nodular pattern and 2 cases had a nodular and diffuse pattern. In all cases, lymphocyte predominant (LP) cells were observed in a background of small lymphocytes and histiocytes. Immunohistochemical analysis showed that the LP cells in all cases were positive for CD20, CD79a, PAX5, OCT2, and CD45 and were negative for CD15. CD30 was expressed variably in 7 cases. EBV encoded RNA was present in all LP cells in 5 cases and in a subset of LP cells in 3 cases. One patient was treated with radiation therapy and 7 patients received chemotherapy, including 4 of 7 patients who underwent autologous stem cell transplantation. EBV infection is a rare primary or secondary event in NLPHL that correlates with poorer prognosis and often requires more aggressive therapy. The variable expression of CD30 in most of these cases could be the result of EBV infection.
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA; Department of Pathology, National University Hospital, Singapore.
Shi
S
Wang
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
L Jeffrey
LJ
Medeiros
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Zijun Y
ZY
Xu-Monette
Department of Pathology and Laboratory Medicine, University of Indiana School of Medicine, IN, USA.
Shanxiang
S
Zhang
Clarient Laboratories, Alisio Viejo, CA, USA.
Dennis P
DP
O'Malley
Department of Pathology, Cornell University School of Medicine, New York, NY, USA.
Attilio
A
Orazi
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Zhuang
Z
Zuo
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Carlos E
CE
Bueso-Ramos
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Cameron C
CC
Yin
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Zhiyu
Z
Liu
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Roberto N
RN
Miranda
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. Electronic address: khyoung@mdanderson.org.
Ken H
KH
Young
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005260:Female; D006689:Hodgkin Disease / Q000473:pathology* / Q000821:virology*; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies
Classical Hodgkin lymphoma; Epstein-Barr virus; Nodular lymphocyte predominant Hodgkin lymphoma