胃と腸54巻13号

2019年12月発行

今月の主題 遺伝子・免疫異常に伴う消化管病変—最新のトピックスを中心に 主題症例

難治性腸管Behçet病として加療中にtrisomy 8陽性の骨髄異形成症候群を合併した1例

著者： 冬野雄太¹ 鳥巣剛弘¹ 平野敦士¹ 梅野淳嗣¹ 藤岡審¹ 森山智彦¹ 江﨑幹宏²

所属機関： ¹九州大学大学院医学研究院病態機能内科学 ²佐賀大学医学部附属病院光学医療診療部

ページ範囲：P.1733 - P.1738

要旨●患者は40歳代，女性．20歳代から回盲部潰瘍，吻合部潰瘍を繰り返し，2度の腸管切除歴があった．30歳代で吻合部潰瘍の再燃のため当科に初診となった．発症16年後に陰部潰瘍が出現し不全型Behçet病と診断された．その後，潰瘍は増悪傾向となり，プレドニゾロン，インフリキシマブなどで加療されたが治療効果に乏しかった．発症19年後，吻合部に下掘れ潰瘍および狭窄を呈し，内科治療に抵抗性であることから吻合部切除術を施行した．しかし，術後早期に潰瘍は再燃増悪し，穿孔を来したため人工肛門造設を要した．術後は在宅中心静脈栄養療法を中心とした加療を行っていたが，発症20年後に汎血球減少を来し，骨髄異形成症候群の合併およびtrisomy 8陽性が確認された．

参考文献

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