Posttransplant Lymphoproliferative Disorder Manifesting
as Intestinal Epstein-Barr Virus-Positive Anaplastic
Large-Cell Lymphoma in an Adult Renal
Transplant Recipient
Pelin Börcek,1 B. Handan Özdemir,1 Gonca Özgün,1 Mehmet Haberal2
Abstract
Introduction
Posttransplant lymphoproliferative disorder is a
relatively common posttransplant malignancy affecting as many as 10% of all solid-organ recipients.
Most cases of posttransplant lymphoproliferative
disorder are of B-cell origin, with common Epstein-Barr
virus association. Posttransplant lymphoproliferative
disorders of T-cell origin are much rarer and less
frequently associated with Epstein-Barr virus. Here, we
report an unusual case of Epstein-Barr virus-positive
anaplastic large-cell lymphoma causing an intestinal
perforation in an adult renal transplant recipient.
A 52-year-old male patient with renal allograft
developed cryptogenic end-stage liver failure and was
accepted as a candidate for liver transplant. Before
transplant, he was admitted with severe abdominal
pain, which turned out to result from ileal perforation.
Pathologic evaluation of the intestinal resection
showed diffuse malignant lymphoid infiltration of the
ileum, consistent with anaplastic large-cell lymphoma.
The tumor was positive for Epstein-Barr virus genome.
Anaplastic large-cell lymphoma is a rare form of
T-cell posttransplant lymphoproliferative disorder
that is infrequently associated with Epstein-Barr
virus. The occurrence of this extraordinary form
of posttransplant lymphoproliferative disorder, its
late onset, intestinal localization, and Epstein-Barr
virus association represent a unique clinical rarity.
Posttransplant lymphoproliferative disorder (PTLD)
is a relatively common posttransplant malignancy
affecting as many as 10% of all solid-organ recipients.
The risk of developing PTLD is highest within the
first year of transplant, and children are more
commonly affected. Of all PTLDs, 85% are of B-cell
origin with over 80% having Epstein-Barr virus
(EBV) association.1 T-cell PTLDs are much rarer and
less frequently associated with EBV.2 Here, we report
a case of EBV-positive anaplastic large-cell lymphoma causing an intestinal perforation in an adult
renal transplant recipient.
Key words: Kidney transplant, T-cell lymphoma
From the 1Department of Pathology, and the 2Department of Transplant Surgery, Başkent
University Faculty of Medicine, Ankara, Turkey
Acknowledgements: The authors declare that they have no sources of funding for this study,
and they have no conflicts of interest to declare.
Corresponding author: Pelin Börcek, Başkent Üniversitesi Patoloji Anabilim Dalı, 79. sokak
(Eski 12. sokak) 7/4, Bahçelievler, Ankara, Turkey 06490
Phone: +90 532 725 4673 E-mail: [email protected]
Experimental and Clinical Transplantation (2016) Suppl 3: 64-66
Copyright © Başkent University 2016
Printed in Turkey. All Rights Reserved.
Case Report
The patient was a 52-year-old male, who had
received a renal allograft transplant 10 years earlier
from his mother due to familial Mediterranean fever.
During the past 8 months, he had experienced
recurrent ascites, complicating his recently diagnosed
cryptogenic liver failure, and the patient was accepted
as a liver transplant candidate. However, the patient
was admitted with severe abdominal pain, shown to
result from ileal perforation. The intestinal resection
specimen showed diffuse and massive wall thickening
on macroscopic evaluation. Microscopically, the
intestinal wall was diffusely infiltrated with large,
pleomorphic and discohesive neoplastic cells (Figure
1), which in some areas took the form of giant cells
(Figure 2). Immunohistochemically, these cells were
positive for cluster of differentiation (CD)45, CD2,
CD30 (Figure 3), and perforin labeling, whereas
CD20, CD3, CD4, CD7, CD8, CD56, anaplastic
lymphoma kinase, granzyme, and CD138 displayed
negative staining. Epstein-Barr-encoded RNA in situ
DOI: 10.6002/ect.tondtdtd2016.P17
Pelin Börcek et al/Experimental and Clinical Transplantation (2016) Suppl 3: 64-66
hybridization was positive (Figure 4). The diagnosis
was monomorphic PTLD manifesting as EBVpositive anaplastic large-cell lymphoma. The patient
died 3 months after diagnosis.
65
Figure 4. Epstein-Barr-Encoded RNA In Situ Hybridization Positivity
(magnification = ×100)
Figure 1. Diffuse Infiltration of the Intestinal Wall by Neoplastic Lymphocytes
(hematoxylin and eosin staining, magnification = ×20)
Discussion
Figure 2. Large, Pleomorphic Tumor Cells With Scattered Giant Cells
(hematoxylin and eosin staining, magnification = ×200)
Figure 3. Immunohistochemical CD30 Positivity (CD30 staining; magnification
= ×100)
Posttransplant lymphoproliferative disorder is a
spectrum of lymphoid proliferations extending from
early, hyperplastic lesions to high-grade lymphomas,
with 85% being of B-cell origin and over 80% of these
having EBV association.1 Posttransplant lymphoproliferative disorders of T-cell origin are rarer and
less frequently associated with EBV.2 A T-cell PTLD
may occur as any T-cell lymphoma listed in World
Health Organization classification, but anaplastic
large-cell lymphoma is the most frequent.3
Posttransplant lymphoproliferative disorders
involve the transplanted organ in over one-half of
cases of heart, lung, and liver transplant recipients.
However, the gastrointestinal system is the most
frequently affected site in renal transplant patients.1
Posttransplant lymphoproliferative disorders are
more common in solid-organ transplant recipients
than in hematologic stem cell recipients, and
many of the cases of intestinal PTLDs reported are of
B-cell phenotype.4 On the other hand, T-cell PTLDs
emerge primarily at extranodal sites, including bone
marrow and spleen, and the small bowel is an
infrequent location. Moreover, the association of
small intestinal T-cell PTLDs with EBV infection is
inconsistent, with only a fraction of cases with EBV
positivity.5
Our case shows numerous clinical and pathological rarities because of the occurrence of PTLD in
an adult patient after a considerably long time gap
after transplant, because of the T-cell phenotype of the
tumor and its intestinal localization, and the relation of
EBV to a T-cell lymphoma.
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Pelin Börcek et al/Experimental and Clinical Transplantation (2016) Suppl 3: 64-66
Conclusions
As the number of end-stage organ disease patients
treated with solid-organ transplant increases, the
incidence of unusual forms of PTLDs may also
increase. The presented case sets a unique example
of this situation.
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