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Emergence of Epstein-Barr virus-associated haemophagocytic syndrome upon treatment of systemic lupus erythematosus

First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan

H Nakamura

First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan, nakamura_hideki911{at}yahoo.co.jp

A Kawakami

First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan

H Ida

First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan

Y Izumi

First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan

M Tamai

First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan

T Origuchi

Nagasaki University School of Health Science, Nagasaki, Japan

M Moriuchi

Division of Medical Virology, Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan

H Moriuchi

Division of Medical Virology, Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan

K Eguchi

First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan

A 32-year-old female patient with systemic lupus erythematosus was admitted to our hospital with fever and cytopenia, and diagnosed as haemophagocytic syndrome (HPS) by bone marrow aspiration study showing haemophagocytosis. Since the serologic activity of lupus was not increased at that time and HPS was refractory to the conventional therapies, an additional aetiological factor was suspected. Real-time PCR analysis identified reactivation of Epstein-Barr virus (EBV). A combination therapy targetting EBV-associated HPS, consisting of intravenous administration of cyclosporine A as well as immunoglobulin with a high titre of anti-EBV antibody, significantly suppressed EBV viraemia and led to the remission of HPS until the time of writing.

Key Words: Epstein-Barr virus • haemophagocytic syndrome • SLE

Lupus, Vol. 15, No. 1, 51-53 (2006)
DOI: 10.1191/0961203306lu2247cr


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