川崎病が疑われ，血球貪食性リンパ組織球症様の病態を示した 急性炎症性疾患の 1 例

Abstract

症例は 7 歳女児．臨床所見から不全型川崎病を疑い免疫グロブリン療法（ intravenous immunoglobulin：IVIG ）を施行され たが，高熱と 2 系統の血球減少が進行し両肺に多発結節影を認めた．血球貪食性リンパ組織球症（ hemophagocytic lymphohistiocytosis：HLH ）も疑われたため，両者に有効なステロイドパルス療法を施行したところ，臨床症状は改善し肺結 節影は消失した．川崎病および HLH は共通する臨床症状，検査所見があり，また両者を合併することもあるため，診断や治療 選択に苦慮することも多い．今回川崎病が疑われ，HLH 様の病態を示した急性炎症性疾患の 1 例を報告する．

A 7-year-old girl was suspected of having incomplete Kawasaki disease. We started treating her with intravenous immunogloblin (IVIG), but her fever and thrombocytopenia progressed and multiple pulmonary nodules were found on her chest computed tomography. Methylprednisolone pulse therapy, which is effective against both Kawasaki disease and hemophagocytic lymphohistiocytosis (HLH) was added because HLH was also suspected. After treatment, there was immediate improvement in her symptoms and the bilateral pulmonary nodular shadows had completely disappeared. Kawasaki disease and HLH have similar symptoms and sometimes the two diseases coexist. It is sometimes difficult to distinguish Kawasaki disease from HLH, and to decide which treatment to use. This was a case of acute inflammatory disease suspicious of incomplete Kawasaki disease that had a clinical course similar to that of HLH.