Abstract
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.
MeSH terms
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Adult
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Antibodies, Viral / analysis
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Biopsy, Needle
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Bone Marrow / pathology
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DNA, Viral / analysis
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Diagnosis, Differential
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Drug Therapy, Combination
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Epstein-Barr Virus Infections / etiology*
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Epstein-Barr Virus Infections / pathology
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Epstein-Barr Virus Infections / virology
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Female
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Follow-Up Studies
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Glucocorticoids / adverse effects*
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Herpesvirus 4, Human / genetics
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Herpesvirus 4, Human / immunology
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Humans
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Immunosuppressive Agents / adverse effects*
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Immunosuppressive Agents / therapeutic use
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Lupus Erythematosus, Systemic / complications
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Lupus Erythematosus, Systemic / drug therapy*
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Lymphohistiocytosis, Hemophagocytic / etiology*
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Lymphohistiocytosis, Hemophagocytic / pathology
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Lymphohistiocytosis, Hemophagocytic / virology
Substances
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Antibodies, Viral
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DNA, Viral
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Glucocorticoids
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Immunosuppressive Agents