演題詳細

一般口演 / Oral Session

一般口演 74 (Oral Session 74) :貧血の臨床

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日程
2013年10月13日(日)
時間
08:45 - 09:45
会場
第4会場 / Room No.4 (さっぽろ芸文館 3F 黎明)
座長・司会
亀岡 淳一 (Junichi Kameoka):1
1:東北大学 血液・免疫病学分野
 
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Long-term outcomes of AA or hypoplastic MDS children who received the same immunosuppressive therapy

演題番号 : OS-3-13

濱 麻人 (Asahito Hama):1、村松 秀城 (Hideki Muramatsu):1、伊藤 雅文 (Masafumi Ito):2、小阪 嘉之 (Yoshiyuki Kosaka):3、土田 昌宏 (Masahiro Tsuchida):4、高橋 義行 (Yoshiyuki Takahashi):1、小林 良二 (Ryoji Kobayashi):5、伊藤 悦朗 (Etsuro Ito):6、矢部 普正 (Hiromasa Yabe):7、大賀 正一 (Shouichi Ohga):8、小原 明 (Akira Ohara):9、小島 勢二 (Seiji Kojima):1

1:Department of Pediatrics, Nagoya University, Nagoya, Japan、2:Department of Pathology, Red Cross Nagoya 1st Hospital、3:Division of Hematology/Oncology, Hyogo Children's Hospital, Kobe, Japan、4:Division of Hematology/Oncology, Ibadaki Children's Hospital, Mito, Japan、5:Department of Pediatrics, Sapporo Hokuyu Hospital, Sapporo, Japan、6:Department of Pediatrics, Hirosaki University, Hirosaki, Japan、7:Department of Cell Transplantation and Regenerative Medicine, Tokai University, Isehara, Japan、8:Department of Pediatrics, Kyushu University, Fukuoka, Japan、9:Department of Transfusion Medicine, Toho University, Tokyo, Japan

 

The 2008 WHO classification proposed a new entity in childhood MDS, refractory cytopenia of childhood (RCC). However, it is not clear whether the morphological classification reflects clinical outcome. We retrospectively reviewed bone marrow morphology in 186 children (median age, 8; range, 1-16 years) who were enrolled in the AA 97 study and received horse ATG and cyclosporine between July 1999 and November 2008. The median follow-up period was 87 months (range, 1-146). Sixty-two (33%) were classified as AA, 94 (49%) as RCC and 34 (18%) as RCMD. After 6 months, the response rates to immunosuppressive therapy were not significantly different among the three groups. Acquisition of chromosomal abnormalities was observed in 5 in AA (monosomy7, n=4; other, n=1), 4 in RCC (monosomy7, n=1; trisomy8, n=1; other, n=2) and 3 patients in the RCMD group (trisomy8, n=3). Although the cumulative incidence (CI) of total clonal evolution at 10 years was not significantly different among the three groups, the CI of monosomy7 was significantly higher in AA than in the other groups (p=0.02). Multivariate analysis revealed that only duration of G-CSF administration of >40 days was a significant risk factor for the development of monosomy7 (p=0.016). Although FFS at 10 years did not significantly differ among the three groups, OS at 10 years in AA (85%) was significantly lower than that in the RCC (97%) and RCMD (100%) groups (p=0.01). These findings suggests that strict morphological differentiation from hypoplastic MDS cannot eradicate the clonal evolution in children with AA.

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