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〉

急性白血病への移行

著者： 松村到¹

所属機関： ¹大阪大学大学院医学系研究科血液・腫瘍内科

ページ範囲：P.271 - P.279

　真性多血症(PV)，本態性血小板血症(ET)，原発性骨髄線維症(PMF)などの骨髄増殖性疾患(MPD)の一部の症例は骨髄異形成症候群(MDS)や急性骨髄性白血病(AML)へと移行する．診断10年後までにAML/MDSに移行する確率は，PVで5～15%，ETで2～5%程度であり，ヒドロキシウレアなどの治療薬の関与を示唆する報告がある．一方，PMFでは10～30%とより高頻度に，より早期からAML/MDSへの移行がみられ，疾患自体の自然経過と考えられる．白血病化した症例の約半数においてMPDの時期にJAK2変異が認められても，AML細胞にはJAK2変異が認められないことから，MPDからの白血病発症にはさらなる遺伝子異常が関与することも示唆されている．

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