Hemophagocytic Syndrome as an Initial Presentation of Systemic Lupus Erythematosus: A Case Report

Hemophagocytic syndrome (HPS) is an uncommon potentially life-threatening hematological disorder characterized by fever, pancytopenia, lymphadenopathy, and activation of macrophages, which can be associated with various diseases. HPS brings significant diagnostic and therapeutic challenges, especially if it is the presenting manifestation of an autoimmune disorder, which is uncommon. We present a case of hemophagocytic syndrome as an initial presentation of systemic lupus erythematosus (SLE). We also highlight this rare initial presentation of SLE where initial antinuclear antibody and extractable nuclear antigen tests were negative. To the best of our knowledge, this is the first case of isolated HPS evolving into SLE in the Middle East.