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Case Reports
. 2024 Oct 22:17:861-867.
doi: 10.2147/IMCRJ.S477296. eCollection 2024.

A Rare Case of Life-Threatening Jaundice Caused by Epstein-Barr Virus Infection and Secondary Cold Agglutinin Syndrome Successfully Treated with Rituximab

Matteo Bellia  1 , Mariangela Greco  1 , Monia Lunghi  1 , Riccardo Moia  1 , Gianluca Gaidano  1 , Andrea Patriarca  1
Affiliations
Case Reports

A Rare Case of Life-Threatening Jaundice Caused by Epstein-Barr Virus Infection and Secondary Cold Agglutinin Syndrome Successfully Treated with Rituximab

Matteo Bellia et al. Int Med Case Rep J. .

Abstract

Background: Jaundice and hyperbilirubinemia are common clinical problems characterized by the presence of bile pigments in the blood and their deposition in body tissues. This clinical condition can be associated with a broad spectrum of potential benign and malignant causes, including hepatic inflammation, biliary obstruction, impaired bilirubin conjugation and bilirubin overproduction Therefore, the hyperbilirubinemia diagnostic work-up sometimes can be highly challenging and its therapeutic management can require a multidisciplinary approach.

Case report: We report on a unique case of life-threatening jaundice and hepatic failure in a 20-year-old female who presented to the emergency room with complaints of fever, constant left abdominal pain and generalized profuse fatigue. A complete and detailed medical history, multiple tests for various infection, radiologic investigations and histological tests were performed in order to clarify the etiology of that rapidly progressive clinical condition. Based on the results, the patient jaundice was caused by an Epstein-Barr virus (EBV) infection and secondary cold agglutinin syndrome. Given the rare and complex diagnosis, multiple clinical specialists were asked to carry out the best patient management.

Conclusion: This rare case highlights how challenging the differential diagnosis and treatment of hyperbilirubinemia can be, presenting a unique case of life-threatening multifactorial hepatic failure treated successfully with rituximab.

Keywords: Epstein-Barr virus infection; jaundice; rituximab; secondary cold agglutinin syndrome.

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Conflict of interest statement

Prof. Dr. Gianluca Gaidano reports personal fees from AbbVie, Astra Zeneca, BeiGene, Hikma, Johnson and Johnson, and Lilly, outside the submitted work. The authors have no other competing interests or other interests that might be perceived to influence the results and/or discussion reported in this paper. No institutional approval is required. All the authors already approved the manuscript. This paper has been uploaded to Authorea as a preprint: https://www.authorea.com/doi/full/10.22541/au.171773811.15814855/v1

Figures

Figure 1
Figure 1
Hematoxylin-Eosin histopathological image of liver biopsy (resolution power 40x) of a 20-year-old female diagnosed with hepatic failure caused by EBV infection and secondary cold agglutinin syndrome. The image reveals a single ring granuloma associated with portal space inflammation and cholestasis. This histopathological pattern is coherent with EBV infection.
Figure 2
Figure 2
Histopathological image of liver biopsy (resolution power 40x) of Epstein-Barr-Virus-Encoded-RNA (EBER) detected by in situ hybridization in a 20-year-old female diagnosed with hepatic failure caused by EBV infection and secondary cold agglutinin syndrome. The image reveals sporadic EBER – positive cellular elements coherent with EBV infection.
Figure 3
Figure 3
Hematoxylin-Eosin histopathological image of bone marrow biopsy (resolution power 40x) of a 20-year-old female diagnosed with hepatic failure caused by EBV infection and secondary cold agglutinin syndrome. The image reveals ring granulomas coherent with EBV infection.
Figure 4
Figure 4
Histopathological image of bone marrow biopsy (resolution power 40x) of Epstein-Barr-Virus-Encoded-RNA (EBER) detected by in situ hybridization in a 20-year-old female diagnosed with hepatic failure caused by EBV infection and secondary cold agglutinin syndrome. The image reveals sporadic EBER – positive cellular elements coherent with EBV infection.
Figure 5
Figure 5
Timeline in days depicting clinical care of a 20-year-old female diagnosed with hepatic failure caused by EBV infection and secondary cold agglutinin syndrome. The day 0 corresponds to patient hospitalization. The variation of patient bilirubin and hemoglobin values are presented in relation to the clinical management.
See this image and copyright information in PMC

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