Bckground Hemophagocytic syndrome (HS) is an hyperinflammatory syndrome caused by severe hypercytokinemia with excessive activation of macrophages and lymphocytes. HS can be primary, or secondary, as a response to infection, malignacy or autoimmune disease.

Aim The description of a case of HS as the first manifection of systemic lupus erythematosus diagnosed at our paediatric department. and arise the awareness of the hospital and general paediatrician for the clinical and laboratory presentation of this rare disease.

Method A nine year girl was admitted, due to fever and fatigue. During her hospitalisation, she developed anaemia and leukocytopenia, elevated liver enzymes and renal function worsening (Urea: 59 mg/dl, microscopic hematuria, albuminuria). Prolonged fever, splenomegaly, bicytopenia, hyperferritinemia (Fer: 38 450 ng/ml) and hypertriglyceridemia (TRGs: 468 mg/dl) put the suspicion of HS diagnosis. Immunology tests revealed the presence of positive anti-nuclear antibodies and low C3 and C4 levels. She was transferred to a tetriary paediatric hospital for further investigation and treatment.

Results At the tertiary paediatric hospital, secondary hemophagocytic syndrome was confirmed -hemophagocytosis was found at a bone marrow puncture-biopsy that was performed- and Systemic Lupus Erythematosus proved to be the primary cause. The duration of hospitalisation was 15 days, and the treatment included methylprednisone, ivig, hydroxychlroroquine and cyclophosphamide.

Conclusion Hemophagocytic syndrome is a rare life threatening entity, that should be taken into concideration when prolonged fever is investigated. Being connected to bad prognosis and risk of permanent tissue damage, prompt diagnosis and treatment are required.