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Int J Clin Exp Pathol 2013;6(11):2620-2624

Case Report
An adult case of systemic Epstein-Barr virus-positive T/natural killer-cell
lymphoproliferative disorder with good outcome

Xiuming Zhang, Zhaoming Wang, Lijun Wang, Hongtian Yao

Department of Pathology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province, China

Received August 18, 2013; Accepted September 15, 2013; Epub October 15, 2013; Published November 1, 2013

Abstract: Epstein-Barr virus-positive T/natural killer (NK)-cell lymphoproliferative disorder (EBV+T/NK LPD) encompasses a heterogeneous
group of disorders that have a common feature with excessive lymphoid proliferation of mainly T cells and/or NK cells. This disease is rare,
predominantly affects children and young adults, and associated with high mortality. Herein, we report a case of EBV+T/NK LPD that occurred
in an old woman with good outcome. The patient presented with fever, splenomegaly, and pancytopenia. Computed tomography (CT) scan of
the abdomen showed splenomegaly. The clinical impression was a malignant tumor of spleen, so splenectomy was performed.
Microscopically, the architecture of the spleen was preserved. The white pulp Malpighian corpuscles were atrophied. The red pulp showed
intact sinusoids and pulp cords with increased cellular infiltrate. The proliferating lymphoid cells were mostly small lymphoid cells with minimal
or no nuclear atypia, mixed with rare medium-sized or large cells. Immunohistochemical study and in-situ hybridization showed that the
EBER-positive lymphoid cells were positive for CD3 and CD56. They were also positive for cytotoxic molecules, such as T-cell restricted
intracellular antigen (TIA1), granzyme B. The case exhibited polyclonal rearrangement of T-cell receptor gene (TCR) by polymerase chain
reaction (PCR) studies. Without radiotherapy and chemotherapy, the patient is alive and well with no evidence of disease 25 months after
surgery. (IJCEP1308048).

Keywords: Epstein-Barr virus, T/natural killer-cell, lymphoproliferative disorder

Address correspondence to: Dr. Zhaoming Wang, Department of Pathology, The First Affiliated Hospital, College of Medicine, Zhejiang
University, No.79 Qingchun Road, Hangzhou, Zhejiang 310003, PR China. Tel: +86-571-87236364; Fax: +86-571-87236570; E-mail:
wangzhaoming1963@163.com