title: [Retrospective analysis of treatment strategy and prognosis in childhood myelodysplastic syndromes from China and Japan].
abstract: This study was aimed to retrospectively analyse the prognosis of childhood myelodysplastic syndromes (MDS-RCC ) from China and Japan. Two hematologists and one pathologist from China and Japan constituted a diagnosis group. According to the criteria of 2008 WHO, 33 children with MDS-RCC from 50 chinese children diagnosed as acquired bone marrow failure syndrome from 2009 to 2011, and 74 Japanese children with MDS-RCC in a prospective registration group conducted by the Japanese Society of Pediatric Hematology were enrolled in this study. The outcome of total 107 childhood MDS-RCC treated with different treatment strategies was analyzed retrospectively. The results indicated that: (1) the 3 and 5-year overall survival rates (OS) for all patients were 93.8% and 79.7% respectively. (2) All 107 patients with MDS-RCC were further subclassified into 2 groups: transfusion dependent group and transfusion independent group. The 3 and 5-year overall survival rates (OS) for transfusion dependent group were 89.9% and for transfusion independent group were 70.6% respectively, the 5-year overall survival rate (OS) for transfusion independent group was 100.0%. (3) Treatment strategy: patients from transfusion dependent group were treated with immunosuppression therapy (IST), in which CsA combined with or without ATG and patients were treated with HSCT. The 5-year overall survival rate (OS) was 100% for IST group. The 3 and 5-year overall survival rates (OS) for HSCT patients were 82.9% and 30.6%, respectively. All the patients from transfusion independent group were alive till the last follow up. (4) Compared with patients from our hospital and Japan, the 5-year overall survival rate (OS) for patients in our hospital was 100.0%, the 3-and 5-year overall survival rates (OS) for patients in Japan were 93.7% and 75.0%, respectively. It is concluded that children with MDS-RCC are seldom progressive. The observation and wait strategy is applicable for patients with MDS-RCC who have no transfusion dependent. IST therapy is recommended to those who have transfusion-dependent.
authors.affiliation: Diagnosis & Treatment Center of Pediatric Blood Diseases, Institue of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences, Tianjin 300020, China.
authors.forename: Xiao-Juan
authors.initials: XJ
authors.lastname: Chen
authors.forename: Atsushi
authors.initials: A
authors.lastname: Manabe
authors.forename: Wen-Yu
authors.initials: WY
authors.lastname: Yang
authors.forename: Pei-Hong
authors.initials: PH
authors.lastname: Zhang
authors.forename: Shu-Chun
authors.initials: SC
authors.lastname: Wang
authors.forename: Ye
authors.initials: Y
authors.lastname: Guo
authors.forename: Fang
authors.initials: F
authors.lastname: Liu
authors.forename: Li-Xian
authors.initials: LX
authors.lastname: Chang
authors.forename: Wei
authors.initials: W
authors.lastname: Wei
authors.forename: Yang
authors.initials: Y
authors.lastname: Wan
authors.forename: Xiao-Fan
authors.initials: XF
authors.lastname: Zhu
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002681:China; D005260:Female; D006801:Humans; D007223:Infant; D007564:Japan; D008297:Male; D009190:Myelodysplastic Syndromes / Q000175:diagnosis* / Q000628:therapy*; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome