A nationwide survey of hypoplastic myelodysplastic syndrome (a multicenter retrospective study)

作者：Kobayashi Takashi; Nannya Yasuhito; Ichikawa Motoshi; Oritani Kenji; Kanakura Yuzuru; Tomita Akihiro; Kiyoi Hitoshi; Kobune Masayoshi; Kato Junji; Kawabata Hiroshi; Shindo Motohiro; Torimoto Yoshihiro; Yonemura Yuji; Hanaoka Nobuyoshi; Nakakuma Hideki; Hasegawa Daisuke; Manabe Atsushi; Fujishima Naohito; Fujii Nobuharu; Tanimoto Mitsune; Morita Yasuyoshi; Matsuda Akira; Fujieda Atsushi; Katayama Naoyuki; Ohashi Haruhiko; Nagai Hirokazu; Terada Yoshiki; Hino Masayuki

来源：American Journal of Hematology, 2017, 92(12): 1324-1332.

DOI：10.1002/ajh.24905

Hypoplastic myelodysplastic syndrome (hMDS) is a distinct entity with bone marrow (BM) hypocellularity and the risk of death from BM failure (BMF). To elucidate the characteristics of hMDS, the data of 129 patients diagnosed between April 2003 and March 2012 were collected from 20 institutions and the central review team of the National Research Group on Idiopathic Bone Marrow Failure Syndromes, and compared with 115 non-hMDS patients. More RA and fewer CMMoL and RAEB-t in French-American-British (FAB) and more RCUD and MDS-U and fewer RCMD in World Health Organization (WHO) classifications were found in hMDS than non-hMDS with significant differences. The overall survival (OS) and AML progression-free survival (AML-PFS) of hMDS were higher than those of non-hMDS, especially in patients at age 50 and of lower risk in Revised International Prognostic Scoring System (IPSS-R). In competing risks analysis, hMDS exhibited decreased risk of AML-progression in lower IPSS or IPSS-R risk patients, and higher risk of death from BMF in patients at age 50. Poor performance status (PS 2) and high karyotype risks in IPSS-R (high and very high) were significant risk factors of death and AML-progression in Cox proportional hazards analysis.

• 出版日期2017-12
• 单位河北医科大学