儿童骨髓增生异常综合征21例临床分析  

Clinical analysis on 21 cases of pediatric myelodysplastic syndrome

作　　者：董南南[1] 徐学聚[1] Dong Nannan;Xu Xueju(Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, Chin)

机构地区：[1]郑州大学第一附属医院儿科,450052

出　　处：《中国实用医刊》2018年第7期51-53,共3页Chinese Journal of Practical Medicine

摘　　要：目的分析儿童骨髓增生异常综合征（MDS）的临床特点、实验室检查、诊断、治疗、转归及预后。方法根据儿童MDS诊断与治疗中国专家共识（2015年版）对21例儿童MDS的临床表现、实验室检查、诊断、治疗、转归及预后进行分析，并对其重新分型。结果21例患儿中男14例，女7例；临床表现：贫血12例（57．1％），出血6例（28．6％），发热15例（71．4％），肝脾肿大11例（52．4％）。实验室检查结果：骨髓增生活跃19例（90．5％）；主要特点为病态造血，单系病态造血4例（19．O％），两系病态造血6例（28．6％），三系病态造血11例（52．4％）；染色体核型异常3例（14．3％）。治疗和转归：5例行造血干细胞移植治疗，6例化疗，1例仅行支持治疗，8例放弃治疗，1例失访；6例患儿转化为急性白血病，转化时间（1～26个月），12例（57．1％）死亡。结论儿童MDS的病程短、全细胞减少、骨髓病态造血、白血病转化率高、病死率高，造血干细胞移植是目前唯一可以治愈的治疗方法。Objective To analyze the clinical features, laboratory examinations, diagnosis, treatment, outcome and prognosis of pediatric myelodysplastic syndrome （MDS）. Methods The clinical features, laboratory features, diagnosis, treatment, outcome and prognosis of the 21 cases were retrospec- tively studied and they were reclassified according to the criterion of ＂2015 edition the Chinese expert consensus for the diagnosis and treatment of pediatric myelodysplastic syndrome＂. Results Of the 21 children, 14 cases were males and 7 cases were females. Clinical symptoms ： there were 12 cases of ane- mia （57.1%） , 6 cases of bleeding （28.6%） , 15 cases of fever （71.4%） , 11 cases of hepatomegaly and splenomegaly （52.4%）. Laboratory examinations： bone marrow proliferation was active in 19 eases （90. 5% ） ; dysplasia was important features in bone marrow, and 4 cases （19. 0% ） had single lineage of hematopoietic dysplasia, 6 cases （28.6%） had two lineages of hematopoietic dysplasia, 11 cases （52. 4% ） had three lineages of hematopoietic dysplasia; 3 cases （14. 3% ） had abnormal chromosome. Treatment and outcomes： 5 cases were treated by hematopoietic stem cell transplantation （HSCT）, 6 ca- ses were treated by chemotherapy, 1 case was given single supportive treatment, 8 cases abandoned treat- ment and 1 case was lost; six children were transformed into acute leukemia, transformation time was 1 -26 months; 12 cases （57.1%） died. Conclusions Pediatric MDS is characterized by short disease history, cytopenia, hematopietic dysplasia, high fatality rate and high rate of conversion to leukemia. HSCT is the only treatment option with a realistic curative potential for pediatric MDS.

关 键 词：骨髓增生异常综合征 儿童 治疗 

分 类 号：R725.5[医药卫生—儿科]