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 〈治療―治療方針・治療成績・予後〉

原発性骨髄線維症

著者： 石澤丈¹ 岡本真一郎¹

所属機関： ¹慶應義塾大学医学部血液内科

ページ範囲：P.293 - P.298

　原発性骨髄線維症は平均生存期間3～5年の難治進行性疾患であり，血球減少に伴う感染症や出血，脾腫，白血化，門脈圧亢進などを主要な臨床像とする病態である．その治療は症状軽減のための対症療法と根治を目的とした同種造血幹細胞移植に大別される．しかし，最近ではJAK2 V617Fに代表されるJAK/STAT経路を恒常的に活性化する遺伝子変異が一部の症例において病態の根幹をなすことが示され，多くの分子標的治療薬が臨床研究の段階に躍進した．本稿では，原発性骨髄線維症における対症療法の意義・同種造血幹細胞移植成績と適応，そして新規治療薬の現状につき概説する．

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