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).
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