Korean J Hematol.  2006 Dec;41(4):311-316. 10.5045/kjh.2006.41.4.311.

A Case of Chronic Active Epstein-Barr Virus Infection with Autoimmnune Hepatitis and a Coronary Aneurysm

• Kim MJ
• Lee JJ
• Park KS
• Kim SY
• Kil HR

• KMID: 2305220
• DOI: http://doi.org/10.5045/kjh.2006.41.4.311

Abstract

Infectious mononucleosis caused by primary infection of Epstein-Barr virus (EBV) is a self-limiting lymphoproliferative disease, and shows concomitant clinical features such as pyrexia, anorexia, sore throat, cervical lymphadenopathy, liver dysfunction and hepatosplenomegaly. In rare cases, EBV establishes a latent infection in B lymphocytes and runs a chronic course and shows infectious mononucleosis-like symptoms repeatedly. This syndrome, named chronic active EBV infection, may trigger an autoimmune disease that mainly affectes the liver and red blood cells, and carries a very poor prognosis. The cardiovascular complications of chronic active EBV infection are very rare and may be associated with coronary arterial disease. This case describes a 5-year-old boy, who developed chronic active EBV infection and was diagnosed as having autoimmune hepatitis with a coronary aneurysm.

Keyword

Chronic active EBV infection; Autoimmune hepatitis; Coronary aneurysm

MeSH Terms

Anorexia
Autoimmune Diseases
B-Lymphocytes
Child, Preschool
Coronary Aneurysm*
Epstein-Barr Virus Infections
Erythrocytes
Fever
Hepatitis*
Hepatitis, Autoimmune
Herpesvirus 4, Human*
Humans
Infectious Mononucleosis
Liver
Liver Diseases
Lymphatic Diseases
Male
Pharyngitis
Prognosis

Figure

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  Fig. 1 Hepatosplenomegaly is noted in the simple abdomen radiography.

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  Fig. 2 EBV was observed in many lymphocytes by in situ hybridization in the cervical lymph node (dark stained cells) (immunochemicalhistostaining, ×400).

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  Fig. 3 Multiple conglomerated lymph nodes are found around both upper internal jugular, mid jugular, lower jugular, posterior triangular, submandibular and submental area (A), supraclavicular area (B), Rt. subcarina, paraesophageal area (C), abdominal aorta, inferior vena cava, celiac axis and mesenteric roots (D).

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  Fig. 4 Echocardiogram revealed normal left ventricular size and contractility without any regional wall motion abnormalities. The aneurysm in the proximal left anterior descending artery (LAD) measured 4.6mm (A) and the one in the proximal right coronary artery (RCA) measured 5.7mm (B).

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