A Case of EBV-associated Hemophagocytic Lymphohistiocytosis with Seizure and Loss of Consciousness / 대한소아신경학회지

ABSTRACT

Hemophagocytic lymphohistiocytosis(HLH) is a rare and fatal disorder in children. Persistent fever, hepatosplenomegaly and pancytopenia are observed in the most cases with the characteristic change of serum triglyceride, fibrinogen, ferritin and LDH level. CNS manifestation were developed in 50-70% of HLH. 20% of cases revealed seizure and irritability at diagnosis. Abnormalities on brain imaging, such as diffuse white matter abnormalities and necrotic area with parenchymal volume loss appeared to roughly parallel the severity of clinical manifestations. In HLH, EBV is the major triggering agent inducing hemophagocytosis as well as the fulminant course of disease. Many cases of EBV-HLH had monoclonal origin and respond well to etoposide-containing regimens. Early induction of an etoposide based regimen is critical factor in securing long-term survival in patients with EBV-HLH. We report a case that 13 year-old female patient with seizure and loss of consciousness was diagnosed as EBV-HLH and treated with protocol HLH-94 consistd of etoposide, dexamethasone, cyclosporin.