Acute lupus haemophagocytic syndrome

A 25 year old female who had been on treatment for Systemic Lupus Erythematosus for the last 12 years presented with high grade non-remitting fever and pleural effusion. She had delivered a full term baby girl and soon developed pancytopenia, markedly raised fibrinogen degradation products, deranged coagulation profile, low serum fibrinogen level, raised liver enzymes and grossly raised serum ferritin. Bone marrow examination revealed reactive haemophagocytosis and megaloblastosis. Autoimmune disorders can lead to reactive haemophagocytic syndrome which is a treatable entity even if it presents with life threatening complications