Hemophagocytic lymphohistiocytosis (HLH) is an immune disorder causing severe inflammation, categorized into primary (familial) and secondary (acquired) types; secondary HLH can occur due to various triggers, including infections like Epstein-Barr virus (EBV).
A 12-year-old girl with childhood-onset systemic lupus erythematosus (SLE) developed fever, seizures, and altered mental status, raising concerns for neuropsychiatric SLE or infections, leading to extensive testing and treatment.
Diagnosis of HLH was confirmed through blood tests showing abnormal cell counts and bone marrow analysis revealing hemophagocytic activity, along with CSF analysis that identified EBV as the