Baylor University Medical Center Proceedings October 2017 - 444

The immunohistochemical proﬁle is
generally positive for B-cell markers
CD20, CD19, CD79a, and PAX-5.
CD10 and BCL6 are usually negative, while MUM1 is commonly
positive. Cases with immunoblastic or plasmablastic features may
lack CD20 expression (5). In situ
hybridization for EBER is positive
and is considered the most important test in diagnosis, with the highest diagnostic sensitivity (2).
EBV+ DLBCL has a poor response to treatment, so rapid detection is a necessity. Detection relies
on clinical suspicion and looking for
b
c
EBV in every case of DLBCL. The
prognosis of EBV+ DLBCL is worse
than that of EBV-negative tumors,
with a median survival of 2 years
(5, 6). Prognosis is worse in patients
70 years or older and in those with
B symptoms. Currently, there is no
uniformly accepted treatment for
EBV+ DLBCL beyond the current
standard therapy for DLBCL (2, 6).
Figure 1. Lymph node examination at autopsy. (a) Sheets of large lymphoid cells with irregular vesicular nuclei and The standard treatment for DLBCL
prominent nucleoli, H&E ×400 (b) B-cell lineage demonstrated by diffuse positive CD20 staining, ×200. (c) Epstein- is the combination of rituximab, a
chimeric anti-CD20 monoclonal
Barr virus involvement demonstrated by in situ hybridization for EBER, ×200.
antibody, with cyclophosphamide,
doxorubicin, vincristine, and prednisone (R-CHOP) (2). Some
According to the 2016 World Health Organization
studies suggest improved prognosis with more intensive regiClassiﬁcation of Tumours of Haematopoietic and Lymphoid
mens, such as combination rituximab, etoposide, prednisolone,
Tissues, EBV+ DLBCL, NOS is diagnosed in apparent imvincristine, cyclophosphamide, and doxorubicin (R-EPOCH)
munocompetent patients, usually over 50 years of age (4). This
(7).
lymphoma was a provisional entity in the 2008 World Health
Organization classiﬁcation, entitled EBV+ DLBCL of the el1. Mandell GL. Principles and Practice of Infectious Diseases, 6th ed., Vol. 2.
derly, but the "elderly" designation was substituted with "not
Philadelphia, PA: Elsevier, 2005.
otherwise speciﬁed" with the recognition that this entity occurs
2. Castillo JJ, Beltran BE, Miranda RN, Paydas S, Winer ES, Butera JN.
in younger patients (4, 5). The NOS designation highlights
Epstein-Barr virus-positive diﬀuse large B-cell lymphoma of the elderly:
that the lymphoma must be excluded from more speciﬁc entiwhat we know so far. Oncologist 2011;16(1):87-96.
ties with neoplastic EBV-positive large B cells, such as lym3. Ok CY, Papathomas TG, Medeiros LJ, Young KH. EBV-positive diﬀuse
large B-cell lymphoma of the elderly. Blood 2013;122(3):328-340.
phomatoid granulomatosis, DLBCL associated with chronic
4. Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, Advani
inﬂammation, and the newly designated entity EBV-positive
R, Ghielmini M, Salles GA, Zelenetz AD, Jaﬀe ES. The 2016 revision
mucocutaneous ulcer (4).
of the World Health Organization classiﬁcation of lymphoid neoplasms.
EBV-positive DLBCL accounts for 8% to 15% of DLBCL
Blood 2016, 127(20):2375-2390.
in the Asian population (2-4). Within Western populations, the
5. Nakamura S, Campo E, Swerdlow S. EBV positive diﬀuse large B-cell
of the elderly. In Swerdlow S, Campo E, Harris NL, Jaﬀee ES, Pileri SA,
percentage is lower (<5%) (2, 3). Some studies found a median
Stein H, Thiele J, Vardiman JW, eds. WHO Classiﬁcation of Tumours of
age of 71 and a slight male predominance. Lymph node involveHaematopoietic and Lymphoid Tissues. Lyon, France: IARC, 2008:243-
ment is seen in about 70% of cases. Microscopically, the lymph
244.
node architecture is eﬀaced and consists of a uniform population
6. Lu TX, Liang JH, Miao Y, Fan L, Wang L, Qu XY, Cao L, Gong QX,
of large cells with extensive necrosis, mitoses, and apoptoses. If
Wang Z, Zhang ZH, Xu W, Li JY. Epstein-Barr virus positive diﬀuse large
B-cell lymphoma predicts poor outcome, regardless of the age. Sci Rep
minimal to no reactive component (small lymphocytes, plasma
2015;5(1):12168.
cells, or histiocytes) is seen, the disease is subclassiﬁed as mono7. Song CG, Huang JJ, Li YJ, Xia Y, Wang Y, Bi XW, Jiang WQ, Huang
morphic. The disease is classiﬁed as polymorphic if a reactive
HQ, Lin TY, Li ZM. Epstein-Barr virus-positive diﬀuse large B-cell
component is present (2, 3). This morphologic subclassiﬁcalymphoma in the elderly: a matched case-control analysis. PLoS One
tion has not been shown to have prognostic implications (3).
2015;10(7):e0133973.
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