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KMID : 0882420060700000363
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Korean Journal of Medicine
2006 Volume.70 No. 0 p.363 ~ p.367
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Successful treatment with intravenous immunoglobulin and etoposide in a systemic lupus erythematosus patient with hemophagocytic syndrome
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Yoon Hyun-Jung
Hwang Seung-Hwan
Park Yong-Wook
Kim Yun-A
Lee Shin-Seok
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Abstract
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Hemophatocytic syndrome shows clinical manifestations of high grade fever, cytopenia and elevated liver enzymes and histologically characterized by systemic proliferation of benign hemophagocytic histiocytes. Viral infection and underlying malignancy, such as malignant lymphoma, are known as the most common cause of hemophagocytic syndrome. Cases of patients with hemophagocytic syndrome associated with systemic lupus erythematosus (SLE) were rarely reported.
We report a successfully treated case of hemophagocytic syndrome in a 45-year-old woman with SLE. She initially manifested with high grade fever, pancytopenia, abnormal liver function tests, and elevated ferritin and triglyceride. Bone marrow aspiration and biopsy showed severe hemophatocytosis by histiocytes. Despite of high doses of prednisolone treatment (60 mg/day), high fever and pancytopenia persisted. The administration of intravenous immunoglobulin and etoposide was added to the prednisolone treatment. 2 weeks later, her fever was subsided and peripheral blood counts were fully recovered. She had no flare during 6 months of follow-up.(Korean J Med 70:S363-S367, 2006)
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KEYWORD
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Hemophagocytic syndrome, Systemic lupus erythematosus, Etoposide
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