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儿童骨髓增生异常/骨髓增殖性疾病临床及生物学特征分析
引用本文:杨文钰,赵玉霞,陈晓娟,王书春,郭晔,陈玉梅,邹尧,刘天峰,竺晓凡.儿童骨髓增生异常/骨髓增殖性疾病临床及生物学特征分析[J].中国小儿血液与肿瘤杂志,2009,14(3).
作者姓名:杨文钰  赵玉霞  陈晓娟  王书春  郭晔  陈玉梅  邹尧  刘天峰  竺晓凡
作者单位:1. 北京协和医学院血液学研究所,血液病医院儿科,中国医学科学院,300020
2. 内蒙古兴安盟人民医院血液科
摘    要:目的探讨儿童骨髓增生异常/骨髓增殖性疾病(MDS/MPD)的临床及生物学特征。方法对我科收治的28例MDS/NPD临床资料进行分析总结。结果临床主要以贫血、出血为首发症状,部分可见肝脾肿大;血常规示两系以上血细胞减少,骨髓中可见病态造血,幼稚细胞比例增高;染色体异常多见,可见毒-7、+8等特异性染色体核型异常。结论儿童MDS/MPD非常少见,占儿童血液系统恶性肿瘤的2%,具有与成人MDS不同的临床表现和生物学特征。

关 键 词:生物学特征  骨髓增生异常/骨髓增殖性疾病  儿童

Analysis of clinical and biological characteristics of myeloplastic and myeloproliferative disorders in children
Yang Wenyu,Zhao Yuxia,Che Xiaojuan,Wang Shuchun,Guo Ye,Chen Yumei,Zou Yao,Liu Tianfeng,Zhu Xiaofan.Analysis of clinical and biological characteristics of myeloplastic and myeloproliferative disorders in children[J].Journal of China Pediatric Blood and Cancer,2009,14(3).
Authors:Yang Wenyu  Zhao Yuxia  Che Xiaojuan  Wang Shuchun  Guo Ye  Chen Yumei  Zou Yao  Liu Tianfeng  Zhu Xiaofan
Abstract:Objective To explore the clinical and biologic characteristics of myeloplastic and myeloproliferative disorders(MDS/MPD) in children.Methods Analysis the clinical datas of 28 cases of MDS/MPD.Results The main symptom is anemia or hemorrhage, splenohepatomegaly can be seen in a part of patients.Biarray cytopenia or pancytopenia can be seen in blood regular test.There are myelodisplastia and excess of blast in bone marrow.We can see chromosome abnormalities and specific chromosome such as-7,+8. Conclusion MDS/MPD is uncommon in children,consitituting 2% of all hematological malignancies.There are different clinical and biological characteristics in adults and children.Hematopoietic stem cell transplantation is the only way of cure this disease.
Keywords:biologic characteristics  Myeloplastic/myeloproliferative disorders  Children
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