전신성홍반성낭창의 첫 임상양상으로 발현된 혈구탐식증후군 1례

Abstract

Hemophagocytic syndrome (HPS) is a clinicopathological entity characterized by proliferation and activation of histiocytes with prominent hemophagocytosis in various organs, including bone marrow, and manifests clinically with high grade fever, cytopenia, hepatosplenomegaly, liver dysfunction and coagulopathy. Many diseases have been reported to be associated with this syndrome, particularly infection and malignant lymphoma. Here, we present a case of systemic lupus erythematosus (SLE) complicated by HPS, and the case was successfully treated with corticosteroid.