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Case ReportOpen Access
Acute Lupus Hemophagocytic Syndrome : Report of a Case and Review of the Literature
Mootsikapun P 
,
Sirijerachai C ,
Chansung K ,
Nanagara R
,
Sirijerachai C ,
Chansung K ,
Nanagara R The 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.
Key word : Acute Lupus Hemophagocytic Syndrome, Hemophagocytic Syndrome, Systemic Lupus
Erythematosus
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.
Key word : Acute Lupus Hemophagocytic Syndrome, Hemophagocytic Syndrome, Systemic Lupus
Erythematosus
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