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Lei et al. Hepatoma Res 2016;2:287-92 Hepatoma Research
DOI: 10.20517/2394-5079.2016.18
www.hrjournal.net
Case Report Open Access

Epstein-Barr virus associated secondary

hemophagocytic lymphohistiocytosis with

an unusual presentation of abdominal

compartment syndrome

Li Lei, Camilla J. Cobb, Jeffrey Cao, Anwar S. Raza
Department of Pathology and Human Anatomy, Loma Linda University Medical Center, Loma Linda, CA 92354, USA

Correspondence to: Dr. Anwar S. Raza, Department of Pathology and Human Anatomy, Loma Linda University Medical Center, 11234 Anderson
Street, Room 2516, Loma Linda, CA 92354, USA. E-mail: ARaza@llu.edu

How to cite this article: Lei L, Cobb CJ, Cao J, Raza AS. Epstein-Barr virus associated secondary hemophagocytic lymphohistiocytosis with an
unusual presentation of abdominal compartment syndrome. Hepatoma Res 2016;2:287-92.
ABSTRACT

Article history: Hemophagocytic lymphohistiocytosis (HLH) is a cytokine storm syndrome caused by an
Received: 03-06-2016 overactive but ineffective immune reaction. Without prompt diagnosis and treatment, HLH is
Accepted: 13-10-2016 life-threatening. However, presenting symptoms are often nonspecific, with fatigue and fever
Published: 21-10-2016 being the most common. A high index of suspicion is therefore critical for early diagnosis
and timely management. A previously healthy, 65-year-old female who initially presented
Key words: with fever and abdominal pain developed abdominal compartment syndrome (ACS) requiring
Hemophagocytic lymphohistiocytosis, decompressive laparotomy on hospital day 6. Intraoperative frozen sections of biopsied liver
abdominal compartment syndrome, showed intense portal lymphohistiocytic infiltrates. Epstein-Barr virus DNA copy numbers
liver biopsy, escalated from 600 copies/ mL after admission to 134,000 copies/mL before death. The
Epstein-Barr virus diagnostic criteria of HLH-2004 were met. Patient expired on hospital day 12. It is important to
raise awareness of ACS being an unusual presentation of HLH. Recent changes in diagnostic
criteria tailored to adult HLH cases are reviewed.

INTRODUCTION according to the World Health Organization classification.
Primary HLH is typically seen in children, and caused
Hemophagocytic lymphohistiocytosis (HLH), as its by mutations inherited in an autosomal recessive
name implies, is featured by the hallmark finding pattern. In contrast, adults tend to have the secondary
of hemophagocytosis in addition to uncontrolled form, which is often triggered by malignancy, infection
lymphohistiocytic proliferation. The exact etiology or autoimmune disorders, with T-cell lymphoma being
remains unknown. A widely accepted explanation is
cytokine storm due to an overactive but ineffective the most common malignancy and Epstein-Barr virus
[1]
immune reaction. (EBV) being the most common infection. A genetic
predisposition has been recognized in some but
HLH is classified into primary and secondary forms not all of the adult cases, even with targeted high-

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