Acute Lupus Hemophagocytic Syndrome : Report of a Case and Review of the Literature

PIROON MOOTSIKAPUN, MD*, CHITIIMA SIRIJERACHAI, MD*, KANCHANA CHANSUNG, MD*, RATANAVADEE NANAGARA,MD*

Affiliation : * Department of Internal Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand.

AbstractThe authors reported a case of systemic lupus erythematosus (SLE) with an unusual presen tation. The patient presented with acute febrile illness along with progressive pancytopenia related to increasing hemophagocytic activity of histiocytes in the bone marrow. Concomitant polyarthritis, myositis, nephritis, high titer of antinuclear factor (1 : 2,560) and positive test for anti-DNA antibody made him fit the diagnostic criteria of SLE. No definite evidence of associated infections was con firmed by bacteriologic, serologic and viral studies. He did not respond to empiric antibiotic therapy but dramatically responded to corticosteroid treatment. Therefore, diagnosis of acute lupus hemophagocytic syndrome was made. The clinical presentation, laboratory diagnosis, and management of the patient are discussed and the literature was reviewed and presented.

Keywords : Acute Lupus Hemophagocytic Syndrome, Hemophagocytic Syndrome, Systemic Lupus Erythematosus

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