Case Report
Epstein–Barr Virus Primary Infection Complicated by
Hemophagocytic Lymphohistiocytosis and Plasmablastic
Lymphoma in a HIV-Negative Patient
Nan Chen , Mike Perez, and Martha Mims
Baylor College of Medicine, 1 Baylor Plaza, Houston, TX 77030, USA
Correspondence should be addressed to Nan Chen; nanc@bcm.edu
Received 9 August 2019; Accepted 19 September 2019; Published 7 October 2019
Academic Editor: Kostas Konstantopoulos
Copyright © 2019 Nan Chen et al. is is an open access article distributed under the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
EBV (Epstein–Barr virus) viremia causes immune dysregulation through various mechanisms, and we are understanding more
that mutations in B, T, and NK (natural killer) cell signaling pathways allow EBV complications such as HLH (hemophagocytic
lymphohistiocytosis) and lymphomas to arise. Here, we report a 20-year-old previously healthy, HIV- (human immunodeficiency
virus-) negative male who presented with fevers, sore throat, and lymphadenopathy (LAD). He was found to have EBV viremia,
pancytopenia, and elevated LFTs (liver function tests) suspicious for HLH. Bone marrow biopsy and elevated IL-2 (interleukin)
receptor confirmed this diagnosis. Additionally, gastric biopsy confirmed diagnosis of plasmablastic lymphoma (PBL), a rare,
aggressive HIV- and EBV-associated lymphoma. Both bone marrow and gastric biopsy showed evidence of EBV. Patients with
EBV complications should have a rigorous workup to characterize the full extent of immune dysregulation including genetic
testing at a high-volume center.
1. Case Presentation
e patient was a healthy 20-year-old M who presented with
3 weeks of intermittent fever, sore throat, and painful bi-
lateral cervical lymph nodes. He went to an outside emer-
gency room 5 days prior to presentation and was diagnosed
with infectious mononucleosis via positive monospot and
discharged with symptomatic treatment. He presented to
our institution for 2 days of hematemesis, melena, jaundice,
and continued fevers. On interview with his family, he had a
healthy childhood without prior hospitalizations and one
healthy younger male sibling.
On physical exam, he was febrile and tachycardic. He had
bilateral cervical lymphadenopathy, decreased breath sounds
in his bilateral lung bases, and hepatosplenomegaly. Initial
laboratory testing showed the following: Hgb (hemoglobin)
12.3 g/dL, WBC (white blood cell) 5.2 k/uL, platelets 68 k/uL,
aspartate aminotransferase 361 U/L, alanine aminotransferase
242 U/L, alkaline phosphatase 332 U/L, total bilirubin
10.7 mg/dL, prothrombin time 34.3 seconds, fibrinogen
95 mg/dL, and d-dimer 6.5 ug/mL. HIV (human immuno-
deficiency virus) and hepatitis panel were negative. CT
(computed tomography) of chest, abdomen, and pelvis
showed diffuse lymphadenopathy in axillary, mediastinal,
hilar, retroperitoneal, and inguinal regions, numerous pul-
monary nodules bilaterally, and hepatosplenomegaly. Over
the following 24 hours, the patient’s clinical condition de-
teriorated, and the following day, he was intubated for
hypoxemic respiratory failure, started on broad-spectrum
antibiotics, and given supportive transfusions. EBV viremia
was confirmed with a viral load of 2 million copies/mL
(Figure 1).
An EGD (esophagogastroduodenoscopy) was performed
for bleeding, revealing multiple friable, superficial ulcers
throughout the distal esophagus and stomach inconsistent
with peptic ulcer disease, and biopsies were collected. With
dropping blood counts, triglycerides of 289 mg/dL and
ferritin of 13,000 ng/mL (Figure 2), there was concern for
hemophagocytic lymphohistiocytosis (HLH), and a marrow
exam was performed along with a left inguinal lymph node
Hindawi
Case Reports in Hematology
Volume 2019, Article ID 7962485, 4 pages
https://doi.org/10.1155/2019/7962485