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 話題

骨髄線維化の機序

著者： 後藤明彦¹ 大屋敷一馬¹

所属機関： ¹東京医科大学内科学第一講座

ページ範囲：P.309 - P.314

1．骨髄線維化とサイトカイン

　BCR-ABL陰性の骨髄増殖性腫瘍(myeloproliferative neoplasms；MPN)ではいずれの病型でも骨髄の線維化を合併しうる．原発性骨髄線維症(primary myelofibrosis；PMF)では細胞増殖期を経て比較的早期から，本態性血小板血症(essential thrombocythemia；ET)や真性多血症(polycythemia vera；PV)では経過中あるいは終末期に骨髄の線維化をしばしば認める．

　この線維化は骨髄へのコラーゲン線維の沈着が主体で，骨新生(骨硬化)と血管新生を伴っている．コラーゲン線維の主要な産生細胞である線維芽細胞は非クローナルであり，機能的には正常であることが証明されている¹⁾．したがってMPNは幹細胞のクローナルな疾患であるが，骨髄線維化は反応性のものであると考えられている．そして，これらの疾患では，線維形成を促進させるサイトカインや血管新生促進性サイトカインの過剰発現がみられること^1,2)から，この反応性の線維化には巨核球や血小板，単球などが産生するサイトカイン，特に，病的造血細胞クローンの巨核球のネクローシスによって放出されるサイトカインによる骨髄内サイトカイン過剰状態が関与していることが示唆されている(図1)．

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