小島 祐毅, 前岡 悦子, 二坂 好美, 福島 侑佳, 橋本 卓典, 佐藤 彩, 内藤 美和, 蓼沼 美砂, 森本 博俊, 湯浅 典博

Yuki KOJIMA, Etuko MAEOKA, Yoshimi NISAKA, Yuka FUKUSHIMA, Takunori HASHIMOTO, Aya SATOU, Miwa NAITOU, Misa TADENUMA, Hirotoshi MORIMOTO, Norihiro YUASA

名古屋第一赤十字病院検査部

Department of Clinical Laboratory, Japanese Red Cross Nagoya Daiichi Hospital

キーワード : veno-occlusive disease, sinusoidal obstruction syndrome, ultrasonography, hematopoietic stem cell transplantation, portal vein

症例は44歳男性，骨髄異形成症候群の治療のため造血幹細胞移植が行われた．第30病日ごろから肝機能異常と体重増加を認め，第40病日には血清ビリルビン値3.8 mg/dl，体重増加5.4％と増悪を認めたため腹部超音波検査（Ultrasonography: US）を施行した．USで肝腫大，腹水貯留を認め，左右門脈枝の逆行性血流を認めたことから遅発性肝中心静脈閉塞症（veno-occlusive disease: VOD）と診断した．Prednisolone，遺伝子組替えトロンボモジュリン，ダナパロイドナトリウムによる治療後，門脈血流は両方向性に改善し，血清ビリルビン値と体重の低下とともに門脈血流は求肝性に正常化したことから，第99病日に退院した．VODの診断は主に臨床症状からなされるが，近年ではUSの有用性が認知されるようになり，USによる血行動態評価がEBMTのVOD診断基準に記載されるようになった．自験例においてもVODの診断，治療効果判定，病態の変化を把握する上で，USによる門脈血流評価が有用であった．

A 44-year-old man underwent hematopoietic stem cell transplantation as treatment for myelodysplastic syndrome. Liver dysfunction and weight gain were observed 30 days after transplantation. Ultrasonography (US) was performed 40 days after transplantation for further evaluation of an elevated serum total bilirubin level (3.8 mg/dL) and weight gain (5.4%). It revealed hepatomegaly, ascites, and hepatofugal flow in the portal vein (PV). Prednisolone, recombinant thrombomodulin, and danaparoid sodium were administered based on a diagnosis of veno-occlusive disease (VOD). Hepatopetal flow of the PV was restored during the course of treatment, and we observed reduction in the patient's serum total bilirubin level and body weight. He was discharged 99 days after transplantation. VOD is usually diagnosed based on a patient's clinical presentation; however, US is useful in such cases and is therefore included in the diagnosis and severity criteria for VOD (the European Society for Blood and Marrow Transplantation, 2016). In the present case, PV evaluation was useful for the accurate diagnosis of VOD and assessment of treatment response.