Retrospective analysis of treatment strategy and prognosis in childhood myelodysplastic syndromes from China and Japan.
10.7534/j.issn.1009-2137.2013.03.025
- Author:
Xiao-Juan CHEN
1
;
Atsushi MANABE
;
Wen-Yu YANG
;
Pei-Hong ZHANG
;
Shu-Chun WANG
;
Ye GUO
;
Fang LIU
;
Li-Xian CHANG
;
Wei WEI
;
Yang WAN
;
Xiao-Fan ZHU
Author Information
1. Diagnosis & Treatment Center of Pediatric Blood Diseases, Institue of Hematology and Blood Disease Hospital, Chinese Academy of Medical Sciences, Tianjin 300020, China.
- Publication Type:Journal Article
- MeSH:
Adolescent;
Child;
Child, Preschool;
China;
Female;
Humans;
Infant;
Japan;
Male;
Myelodysplastic Syndromes;
diagnosis;
therapy;
Prognosis;
Retrospective Studies;
Treatment Outcome
- From:
Journal of Experimental Hematology
2013;21(4):940-947
- CountryChina
- Language:Chinese
-
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.
