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Permalink : https://petit.lib.yamaguchi-u.ac.jp/4702
山口医学 Volume 54 Issue 5
published_at 2005-10-31
Three cases of Philadelphia chromosome negative myelodysplastic/myeloproliferative diseases
フィラデルフィア染色体陰性骨髄異形成症候群/骨髄増殖性疾患の3症例
Tanaka Yoshinori
Matsui Kumiko
Sakuragi Shizu
Mitani Noriyuki
Matsuda Kazuhiro
Shinohara Kenji
About This Article
Descriptions
WHO分類により骨髄異形成症候群/骨髄増殖性疾患myelodysplastic/myeloproliferative diseases (MDS)/(MPD)に分類されるPhiladelphia (Ph)染色体陰性の非定型慢性骨髄性白血病atypical CML (aCML) 2例と慢性骨髄単球性白血病chronic myelomonocytic leukemia (CMML) 1例を経験した.aCMLの2症例は白血球増加, 各成熟段階の顆粒球系細胞の増加, 顆粒球の異形成を認めたが染色体異常は認めなかった.CA (cytosine arabinoside, aclacinomycin)療法, interferon (IFN)-a, hydroxyurea (HU)にて治療を行うも不応性であり脳出血を起こし死亡した.CMMLの症例は当初は骨髄線維化を伴うMDSとして発症したが後に著明な単球増加を伴うCMMLへと病型移行した.CA療法, VP-16を投与するも効果なく肺真菌感染症にて死亡した.形態学のみでこれらの疾患の診断を行うのは完全ではない.将来的には分子生物学的異常が解明され, それに基づく診断と特異的治療法の開発が望まれる.
Creator Keywords
骨髄異形成症候群
骨髄増殖性疾患
非定型慢性骨髄性白血病
慢性骨髄単球性白血病
Rights
本文データは山口大学医学会の許諾に基づきCiNiiから複製したものである
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