Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2024 Dec 30;16(12):e76604.
doi: 10.7759/cureus.76604. eCollection 2024 Dec.

Epstein-Barr Virus-Induced Hemophagocytic Lymphohistiocytosis: A Case Report of a Rare and Life-Threatening Syndrome

Usamah Al-Anbagi  1 , Abdulqadir J Nashwan  2 , Claret Charles Isabirye  1
Affiliations
Case Reports

Epstein-Barr Virus-Induced Hemophagocytic Lymphohistiocytosis: A Case Report of a Rare and Life-Threatening Syndrome

Usamah Al-Anbagi et al. Cureus. .

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome of excessive immune activation, leading to severe inflammation and organ damage. While more common in infants, HLH can occur at any age and is often triggered by infections such as Epstein-Barr virus (EBV). In this case, a 38-year-old man presented with a three-week history of fevers, night sweats, poor appetite, and severe anemia. Investigations revealed hepatosplenomegaly, extremely elevated ferritin levels, triglycerides, and a positive EBV PCR. Despite treatment, his condition deteriorated, and a bone marrow biopsy confirmed HLH. He received immunosuppressive therapy but ultimately passed away after 64 days in the hospital. This case emphasizes the diagnostic challenges of HLH, particularly when triggered by the EBV. Early diagnosis and prompt treatment are vital, although prognosis can be poor in severe cases, underscoring the importance of clinical vigilance.

Keywords: allogeneic hematopoietic cell transplant (hct); bone marrow biopsy; cytopenia; epstein-barr virus (ebv); etoposide; ferritin; hemophagocytic lymphohistiocytosis (hlh); hypertriglyceridemia.

PubMed Disclaimer

Conflict of interest statement

Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. The Medical Research Center at Hamad Medical Corporation issued approval MRC-04-24-767. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. The MRI of the abdomen revealed hepatomegaly with hemosiderosis (red arrow), hepatic hemangioma (blue arrow), and splenomegaly with hemorrhagic infarcts (green arrow).
MRI: magnetic resonance imaging
Figure 2
Figure 2. The PET scan revealed diffuse increased metabolic activity in the spleen (blue arrow) and diffuse bone marrow activation or infiltration (red arrows).
PET scan: positron emission tomography scan
See this image and copyright information in PMC

References

    1. Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: recommendations from the North American Consortium for Histiocytosis (NACHO) Jordan MB, Allen CE, Greenberg J, et al. Pediatr Blood Cancer. 2019;66:0. - PMC - PubMed
    1. Histiocytic disorders: recent insights into pathophysiology and practical guidelines. Filipovich A, McClain K, Grom A. Biol Blood Marrow Transplant. 2010;16:0–9. - PubMed
    1. Perforin gene defects in familial hemophagocytic lymphohistiocytosis. Stepp SE, Dufourcq-Lagelouse R, Le Deist F, et al. Science. 1999;286:1957–1959. - PubMed
    1. Viral infections associated with haemophagocytic syndrome. Maakaroun NR, Moanna A, Jacob JT, Albrecht H. Rev Med Virol. 2010;20:93–105. - PMC - PubMed
    1. HLH associated with disseminated tuberculosis. Kessler M, Reinig E. N Engl J Med. 2020;382:1749. - PubMed

Publication types

LinkOut - more resources