ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) can develop following strong activation of the immune system and the cardinal symptoms are a prolonged fever, hematological abnormalities, hepatosplenomegaly, and hemophagocytosis. HLH can be classified as primary or secondary HLH, associated with infections, malignancy and autoimmune disease. There is no consensus on the primary treatment regimen in systemic lupus erythematosus (SLE)-associated HLH. We experienced a case of SLE-associated HLH in a previously healthy adult. She was initially treated with intravenous immunoglobulin, cyclosporine, and high-dose steroid, but had a poor clinical response. After intravenous etoposide, the patient stabilized and has been followed for 1 year without reactivation of the HLH or SLE.