A nationwide survey of hypoplastic myelodysplastic syndrome (a multicenter retrospective study)期刊界 All Journals 搜尽天下杂志传播学术成果专业期刊搜索期刊信息化学术搜索

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

Authors:	Takashi Kobayashi Yasuhito Nannya Motoshi Ichikawa Kenji Oritani Yuzuru Kanakura Akihiro Tomita Hitoshi Kiyoi Masayoshi Kobune Junji Kato Hiroshi Kawabata Motohiro Shindo Yoshihiro Torimoto Yuji Yonemura Nobuyoshi Hanaoka Hideki Nakakuma Daisuke Hasegawa Atsushi Manabe Naohito Fujishima Nobuharu Fujii Mitsune Tanimoto Yasuyoshi Morita Akira Matsuda Atsushi Fujieda Naoyuki Katayama Haruhiko Ohashi Hirokazu Nagai Yoshiki Terada Masayuki Hino Ken Sato Naoshi Obara Shigeru Chiba Kensuke Usuki Masatsugu Ohta Osamu Imataki Makiko Uemura Tomoiku Takaku Norio Komatsu Akira Kitanaka Kazuya Shimoda Kenichiro Watanabe Kaoru Tohyama Akifumi Takaori‐Kondo Hideo Harigae Shunya Arai Yasushi Miyazaki Keiya Ozawa Mineo Kurokawa for National Research Group on Idiopathic Bone Marrow Failure Syndromes

Affiliation:	1. Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan;2. Department of Hematology and Oncology, Graduate School of Medicine, Osaka University, Osaka, Japan;3. Department of Hematology and Oncology, Nagoya University Graduate School of Medicine, Nagoya, Japan;4. Department of Hematology, Fujita Health University School of Medicine, Aichi, Japan;5. Department of Medical Oncology and Hematology, Sapporo Medical University School of Medicine, Sapporo, Japan;6. Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, Kyoto, Japan;7. Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan;8. Oncology Center, Asahikawa Medical University Hospital, Asahikawa, Japan;9. Department of Transfusion Medicine and Cell Therapy, Kumamoto University Hospital, Kumamoto, Japan;10. Department of Hematology/Oncology, Wakayama Medical University, Wakayama, Japan;11. Department of Pediatrics, St. Luke's International Hospital, Tokyo, Japan;12. Division of Blood Transfusion, Akita University Hospital, Akita, Japan;13. Department of Hematology and Oncology, Okayama University Hospital, Okayama, Japan;14. Division of Hematology and Rheumatology, Department of Internal Medicine, Kindai University Faculty of Medicine, Osaka‐Sayama, Japan;15. Department of Hemato‐Oncology, Saitama International Medical Center, Saitama Medical University, Hidaka, Saitama, Japan;16. Department of Hematology and Oncology, Mie University Graduate School of Medicine, Tsu, Mie, Japan;17. Clinical Research Center, National Hospital Organization Nagoya Medical Center, Nagoya, Japan;18. Department of Hematology, National Hospital Organization Nagoya Medical Center, Nagoya, Japan;19. Hematology, Graduate School of Medicine, Osaka City University, Osaka, Japan;20. Division of Hematology, Department of Internal Medicine, National Defense Medical College, Saitama, Japan;21. Department of Hematology, Faculty of Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan;22. Department of Hematology, NTT Medical Center Tokyo, Tokyo, Japan;23. Department of Hematology, Fukushima Medical University Aizu Medical Center, Fukushima, Japan;24. Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Kagawa, Japan;25. Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan;26. Department of Gastroenterology and Hematology, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan;27. Department of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan;28. Department of Laboratory Medicine (Laboratory Hematology), Kawasaki Medical School, Okayama, Japan;29. Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Japan;30. Department of Hematology, Atomic Bomb Disease and Hibakusha Medicine Unit, Atomic Bomb Disease Institute, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan;31. Division of Hematology, Jichi Medical University, Tochigi, Japan;32. IMSUT Hospital, The Institute of Medical Science, The University of Tokyo, Tokyo, Japan

Abstract:	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.

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