Hemophagocytic syndrome diagnosed by liver biopsy in a female patient with systemic lupus erythematosus.

Hemophagocytic syndrome (HPS) is a rare but potentially fatal disease caused by the activation of benign macrophage in various organs including bone marrow and the mononuclear phagocytes system. Here we report a case of 23-year-old woman in whom HPS occurred along with lupus flare during follow-up after the diagnosis of systemic lupus erythematosus. The patient showed aggravated malar rash, discoid rash, febrile cytopenia, hyperferritinemia, and elevated liver enzymes. She underwent ultrasound-guided percutaneous gun biopsy of the liver using an 18-gauge needle. As activated macrophages with hemophagocytosis were confirmed in hepatic histology, the patient was diagnosed as having reactive HPS related to a lupus flare. Clinical symptoms and laboratory findings were improved through high-dose intravenous methylprednisolone therapy followed by oral prednisolone and cyclosporine maintenance therapy. In lupus patients with persistent high fever, cytopenia, elevated levels of serum ferritin, and liver enzymes, reactive HPS should be strongly suspected, and aggressive examinations such as liver biopsy need to be considered for early diagnosis and proper treatment.