Unusual case of adult hemophagocytic syndrome

Abstract

Hemophagocytic syndrome (HPS) is an uncommon manifestation in systemic lupus erythematosus (SLE). Clinical features of HPS include fever, pancytopenia, abnormal liver enzyme, hepatosplenomegaly, lymphadenopathy, and coagulation disorder. HPS comprises primary and reactive forms. Herein, we describe a case of untreated SLE with HPS as one of the first manifestations of systemic Lupus.

Key words: Autoimmunity, hemophagocytic syndrome, hemophagocytosis, systemic lupus erythematosus