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Titolo:
Myelodysplastic syndrome in childhood: a retrospective study of 189 patients in Japan
Autore:
Sasaki, H; Manabe, A; Kojima, S; Tsuchida, M; Hayashi, Y; Ikuta, K; Okamura, J; Koike, K; Ohara, A; Ishii, E; Komada, Y; Hibi, S; Nakahata, T;
Indirizzi:
Kawasaki Kyodo Hosp, Dept Pediat, Kawasaki Ku, Kawasaki, Kanagawa 2100833,Japan Kawasaki Kyodo Hosp Kawasaki Kanagawa Japan 2100833 anagawa 2100833,Japan Univ Tokyo, Inst Med Sci, Tokyo, Japan Univ Tokyo Tokyo JapanUniv Tokyo, Inst Med Sci, Tokyo, Japan Nagoya Univ, Sch Med, Dept Pediat, Nagoya, Aichi 466, Japan Nagoya Univ Nagoya Aichi Japan 466 Dept Pediat, Nagoya, Aichi 466, Japan Childrens Hosp, Ibaraki, Osaka, Japan Childrens Hosp Ibaraki Osaka JapanChildrens Hosp, Ibaraki, Osaka, Japan Univ Tokyo, Sch Med, Dept Pediat, Tokyo 113, Japan Univ Tokyo Tokyo Japan 113 Tokyo, Sch Med, Dept Pediat, Tokyo 113, Japan Yokohama City Univ, Sch Med, Dept Blood Transfus, Yokohama, Kanagawa 232, Japan Yokohama City Univ Yokohama Kanagawa Japan 232 ohama, Kanagawa 232, Japan Shinshu Univ, Sch Med, Dept Pediat, Matsumoto, Nagano 390, Japan Shinshu Univ Matsumoto Nagano Japan 390 iat, Matsumoto, Nagano 390, Japan Mie Univ, Sch Med, Dept Pediat, Tsu, Mie, Japan Mie Univ Tsu Mie JapanMie Univ, Sch Med, Dept Pediat, Tsu, Mie, Japan Kyoto Prefectural Univ, Sch Med, Dept Pediat, Kyoto 606, Japan Kyoto Prefectural Univ Kyoto Japan 606 ed, Dept Pediat, Kyoto 606, Japan Kyoto Univ, Sch Med, Dept Pediat, Kyoto 606, Japan Kyoto Univ Kyoto Japan 606 Univ, Sch Med, Dept Pediat, Kyoto 606, Japan
Titolo Testata:
LEUKEMIA
fascicolo: 11, volume: 15, anno: 2001,
pagine: 1713 - 1720
SICI:
0887-6924(200111)15:11<1713:MSICAR>2.0.ZU;2-I
Fonte:
ISI
Lingua:
ENG
Soggetto:
SEVERE APLASTIC-ANEMIA; JUVENILE MYELOMONOCYTIC LEUKEMIA; BONE-MARROW TRANSPLANTATION; ACUTE MYELOID-LEUKEMIA; SCORING SYSTEM; CHILDREN; MONOSOMY-7; NEUROFIBROMATOSIS; EPIDEMIOLOGY; DELINEATION;
Keywords:
myelodysplastic syndrome; children; clinical features; predisposing conditions; cytogenetics; prognosis;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
32
Recensione:
Indirizzi per estratti:
Indirizzo: Sasaki, H Kawasaki Kyodo Hosp, Dept Pediat, Kawasaki Ku, 2-1-5 Sakuramoto,Kawasaki,Kanagawa 2100833, Japan Kawasaki Kyodo Hosp 2-1-5 Sakuramoto Kawasaki Kanagawa Japan 2100833
Citazione:
H. Sasaki et al., "Myelodysplastic syndrome in childhood: a retrospective study of 189 patients in Japan", LEUKEMIA, 15(11), 2001, pp. 1713-1720

Abstract

We report a retrospective analysis of children with myelodysplastic syndrome (MDS) diagnosed between 1990 and 1997 in Japan. In total, 189 patients were enrolled: 122 cases of primary MDS (26 RA, 18 RAEB, 25 RAEBt, 53 CMML/JMML), 24 cases with constitutional predisposition to MDS, and 43 cases of therapy-related MDS (t-MDS). The frequency of pediatric MDS was estimated tobe 7.7% of all leukemias. Cytogenetic abnormalities were observed in 41% of primary MDS and 90% of t-MDS cases. The 4-year survival rate, estimated by Kaplan-Meier analysis, for primary RA was 78.9%, while other types of MDSand JMML had rates lower than 40%, and t-MDS showed an even more unfavorable prognosis. In primary MDS, the survival rate of patients with cytogenetic abnormalities was significantly lower. Among prognostic variables by IPSS, only the cytogenetic pattern was useful for predicting outcome in childhood MDS. There was no apparent advantage to chemotherapy for RA, and the survival rate In patients with primary RA, JMML, or t-MDS receiving stem cell transplantation was significantly higher. More precise designs of our diagnostic and classification systems, as well as therapeutic trials In large-scale prospective studies, are necessary for further Improvements in MDS outcome.

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Documento generato il 24/01/20 alle ore 16:06:06