Hemophagocytic lymphohistiocytosis associated with sodium valproate

Hemophagocytic lymphohistiocytosis(HLH) is known to have numerous causes, such as chronic inflammation, infection, malignancy, drug use, and primary or familial HLH. HLH triggered by valproate (VPA) has rarely been reported in the literature. Here we describe a pediatric patient with HLH induced by VPA. A 5-years-old girl presented to our center with recurrent fever accompanied by diffuse generalized rash after 2 weeks of taking oral VPA. Physical examination revealed hepatosplenomegaly; laboratory findings showed bicytopenia (hemoglobin and platelet), hemophagocytic cells on the bone marrow smear, hypofibrinogenaemia and hypertriglyceridaemia, and a high ferritin level. She was diagnosed to have HLH associated with VPA. She was treated with intravenous immunoglobulin, glucocorticoid and withdrawal of the sodium valproate, and she completely recovered. In conclusion, VPA can trigger HLH, a potentially fatal condition.