臨床検査54巻3号

2010年03月発行

今月の主題 骨髄増殖性疾患 各論 〈病態―新しい研究の展開〉

原発性骨髄線維症

著者： 竹中克斗¹

所属機関： ¹九州大学大学院医学研究院病態修復内科学

ページ範囲：P.261 - P.270

　原発性骨髄線維症は，造血幹細胞レベルで生じた遺伝子異常により，クローナルな骨髄系細胞増殖をきたし，異常クローン由来の巨核球や単球などからの増殖因子・サイトカインの産生によって骨髄間質細胞の増殖から，骨髄の広範な線維化，骨硬化，血管新生などを生じる．その結果，無効造血や，末梢血での涙滴状赤血球の出現，白赤芽球症，髄外造血による巨脾などの臨床症状を呈する．2005年に骨髄増殖性腫瘍において共通のJAK2チロシンキナーゼの遺伝子変異による活性化が報告され，疾患の本態の解明が急速に進んでいる．疾患の進展につながる分子細胞学的な病態解明が，今後の分子標的療法などの有効な治療法の開発につながると期待される．

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