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増刊号 消化管悪性リンパ腫2014
主題 消化管原発low-grade lymphoma―濾胞性リンパ腫とマントル細胞リンパ腫
文献概要
マントル細胞リンパ腫（mantle cell lymphoma ; MCL）は全非Hodgkinリンパ腫の3～6％を占めるB細胞リンパ腫であり，aggressiveな経過を示すことが多いが，一部に緩徐な経過を示すタイプがあることが知られている．CHOP療法での生存期間中央値は3～5年であるが，リツキシマブ，高用量シタラビン，自家造血幹細胞移植併用の大量化学療法を組み込んだ強力な化学療法の導入によって，65歳以下の若年初発MCLの生存は明らかに改善してきた．自家造血幹細胞移植が非適応である66歳以上の高齢初発MCLでは，R-CHOP療法後のリツキシマブ維持療法が生存改善をもたらすことが検証されている．しかし，適応が限定され，高率の治療関連死亡率を伴う同種移植以外では，MCLに対して治癒をもたらす標準治療は未確立であり，最近の治療戦略でも生存期間中央値は4～6年である．最近，MCLの分子生物学の進歩により，ベンダムスチン，プロテアソーム阻害薬のボルテゾミブ，BTK（Bruton's tyrosine kinase）阻害薬のイブルチニブなど，MCLに対する極めて有望な新薬の開発が進み，MCLのさらなる治療進歩が期待されている．本稿では，MCLの臨床的特徴，診断，標準的治療，新薬の開発動向について述べる．
参考文献
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