경련과 의식소실을 주소로 내원한 Estein-Barr 바이러스 연관성 혈구탐식성 림프조직구증 1례

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

저자들은 두통과 구토, 지속되는 발열을 주소로 내원하여 뇌수막염을 진단받고 치료받던 중 어지럼증, 경부림프절 종창, 홍역모양 홍반, 범혈구 감소증, 간효소 상승과 의식소실을 동반한 경련이 발생하여 뇌자기공명여상과 골수 조직검사 등을 시행하였고 EBV-연관성 혈구탐식성 림프조직구증으로 진단하에 HLH-94 protocol에 따라 면역화학요법으로 치료하여 관해와 함께 신경학적 증상이 호전된 13세된 여아 환자를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

Hemophagocytic lymphohistiocytosis(HLH) is a rare and fatal disorder in children. Per-sistent 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 irri-tability at diagnosis. Abnormalities on brain imaging, such as diffuse white matter 뮤-normalities and necrotic area with parenchymal volume loss appeared to roughly parallel the severity of clinical manifestations. In HLH, EBV is the major triggering agent in-ducing 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.