images and diagnosis

Composite angioimmunoblastic T-cell

and diffuse large B-cell lymphoma
Anna Alperovich a, Panagiotis J Vlachostergios a,*
, Adam Binder b, Andrew H Oliff c,
Rajeev L Balmiki d, Francois Dufresne a
a
Department of Medicine, Lutheran Medical Center, Brooklyn, NY, USA, b Department of Medicine, Icahn School of Medicine, Mount
Sinai Medical Center, New York, NY, USA, c Department of Pathology and Laboratory Medicine, Lutheran Medical Center, Brooklyn, NY, USA,
d
Department of Hematology/Oncology, Lutheran Medical Center, Brooklyn, NY, USA
* Corresponding author at: Department of Medicine, Lutheran Medical Center, 150 55th Street, Brooklyn, NY 11220, USA. Tel.: +1 718 630
6345; fax: +1 718 210 5306. Æ pvlachostergios@lmcmc.com Æ Received for publication 12 March 2015 Æ Accepted for publication 11 April
2015

Hematol Oncol Stem Cell Ther 2015; 8(3): 136–137

ª 2015 King Faisal Specialist Hospital & Research Centre. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND
license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
DOI: http://dx.doi.org/10.1016/j.hemonc.2015.04.006

A
58-year-old female smoker with a history of
hypertension presented to the emergency
department with a recurrent episode of wors-
ening shortness of breath over five days. Her exam
was significant for sinus tachycardia, cervical lym-
phadenopathy, and a firm left supraclavicular node.
Laboratory values demonstrated a normal white cell
count and differential, normocytic anemia, thrombo-
cytopenia, and a lactate dehydrogenase of 803 IU/L.
She was HIV negative. Computerized tomography
(CT) scan of the chest/abdomen/pelvis demonstrated
extensive bilateral cervical, supraclavicular and axillary
adenopathy, mediastinal and hilar lymphadenopathy
with infiltrate and consolidation of the right lower
and posterior upper lobe and encasement of the right
pulmonary artery, as well as splenomegaly with para-
aortic and retroperitoneal adenopathy. An echocar-
diogram revealed a normal left ventricle and a severe
pericardial effusion. She underwent a pericardial win-
dow and an excisional supraclavicular lymph node
biopsy, which revealed T-cells with features of
angioimmunoblastic T-cell lymphoma (AITL) and
large atypical B-cells consistent with diffuse large B-
cell lymphoma (DLBCL) (Figure 1A). Destruction
of the lymph node architecture was noted, with many
medium-sized atypical lymphocytes with irregular
nuclei and moderate amounts of pale cytoplasm. In
addition, scattered and focally increased large
atypical lymphocytes with prominent nucleoli were
seen. Blood vessels were increased. Immunostains
demonstrated atypical T-cells expressing CD3
(Figure 1B), CD4 (Figure 1C), CD5, CD2, CD7,
CD4, CD10, PD-1 (Figure 1D), as well as clustered
large B-cells positive for expression of CD20, Bcl-6
and BOB-1 (Figure 1E). Polymerase chain reaction
(PCR) testing revealed a clonal TCR gamma gene
rearrangement, as well as a clonal IgH gene rearrange-
ment. Epstein-Barr virus-encoded RNA (EBER) was
negative by in situ hybridization (ISH). Bone marrow
biopsy and CSF analysis were negative for lymphoid
or plasma cell infiltrates. The patient was initiated
on R-CHOEP (rituximab, cyclophosphamide, dox-
orubicin, vincristine, etoposide, prednisone) and com-
pleted three cycles of chemotherapy. Her CT scan
after three cycles was consistent with a partial
response, with significantly diminished bilateral axil-
lary lymphadenopathy, improved mediastinal, perihi-
lar and right lower lobe infiltrates, decreased
splenomegaly, and upper abdominal and retroperi-
toneal adenopathy. The patient eventually had pro-
gression of her disease during the course of
subsequent R-CHOEP cycles. She then progressed
through two cycles of gemcitabine-carboplatin and
one course of romidepsin. Her performance status
deteriorated, not enabling any further treatment and
the patient finally expired, approximately 24 months
after initial diagnosis.
This case illustrates a rare hematologic malignancy
presenting with a dual primary lymphoma population.
The treatment of composite lymphoma with simulta-
neous B- and T-cell components is challenging and
the less favorable prognosis component determines

136 Hematol Oncol Stem Cell Ther 8(3) Third Quarter 2015
COMPOSITE AITL AND DLBCL images and diagnosis

Figure 1. (A) At high power (40·), scattered and focally increased large atypical lymphocytes with prominent nucleoli are seen. (B) CD3 stain (pan
T-cell marker) highlights the atypical T-cells. (C) Most have phenotype of CD4 + AITL helper atypical T-cells. (D) PD-1 highlights the T-cells of AITL.
(E) BOB-1 highlights the scattered and clustered large atypical B-cells consistent with the large B-cell lymphoma component.

the therapeutic strategy.1,2 When a T-cell lymphoma
accompanies a treatment-requiring B-cell lymphoma,
an anti-CD20 antibody-containing protocol with
good activity in both entities should be selected.2 In
our case, R-CHOEP was chosen based on previously
shown survival benefit in patients with DLBCL.3 Not
unexpectedly, the outcome is largely determined by
the T-cell component.2 Once in remission, autologous
stem cell transplantation is a suggested strategy, given
that current standard chemotherapy is inadequate for
most patients with aggressive T-cell lymphomas.4
Thus, although initial chemotherapy is relatively more
effective in composite lymphomas than in conven-
tional peripheral T-cell lymphomas, most composite
AITL and DLBCL cases follow an aggressive clinical
course and portend poor prognosis.1
CONFLICT OF INTEREST STATEMENT
None declared.

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2. Kppers R, Dhrsen U, Hansmann ML.
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Pedersen LM, Bukh A, et al. R-CHOEP-14 improves patients with aggressive peripheral T-cell lymphoma
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