中国当代儿科杂志
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中国当代儿科杂志  2015, Vol. 17 Issue (1): 15-21    DOI:10.7499/j.issn.1008-8830.2015.01.004
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儿童难治性血细胞减少的临床特征分析
安文彬1, 张培红2, 任媛媛1, 郭晔1, 王书春1, 陈晓娟1, 杨文钰1, 常丽贤1, 竺晓凡1
1. 中国医学科学院北京协和医学院血液病医院血液学研究所 儿童血液病诊疗中心, 天津 300020;
2. 中国医学科学院北京协和医学院血液病医院血液学研究所 血液病理诊断中心, 天津 300020
Clinical features of childhood refractory cytopenia
AN Wen-Bin1, ZHANG Pei-Hong2, REN Yuan-Yuan1, GUO Ye1, WANG Shu-Chun1, CHEN Xiao-Juan1, YANG Wen-Yu1, CHANG Li-Xian1, ZHU Xiao-Fan1
Pediatric Blood Diseases Centre, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China
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摘要 目的 研究儿童难治性血细胞减少(RCC)的临床特征.方法 回顾性分析1990 年1 月到2013 年6 月1 420 例初诊为非重型再生障碍性贫血儿童(0~14 岁)的临床资料,重新评估患儿骨髓细胞形态和组织病理,按照2008 版WHO 造血与淋巴组织肿瘤中RCC 诊断标准重新进行诊断分型.患儿临床转归通过每3~6 月门诊就诊随访获得.结果 1 420 例患儿中,152 例(10.7%)诊断为RCC.与非重型再生障碍性贫血患儿比较,RCC 患儿发病时血红蛋白较低,胎儿血红蛋白比例增高.RCC 患儿诊断时异常核型者比例为21.5%.中位随访时间为36 个月(范围1~283 个月).RCC 患儿对环孢素和雄激素治疗的完全有效、部分有效和无效率分别为19.0%、26.7%、54.3%.RCC 患儿5 年和10 年的预期生存率分别为87.9% 和72.4%;5 年和10 年的预期克隆演变率分别为15.3% 和20.0%;2 年预期新发染色体核型异常率为3.6%;5 年和10 年预期白血病转化率为10.0% 和20.0%.结论 RCC 具有成人骨髓增生异常综合征的基本特征.RCC 患儿预后差,向白血病转化风险高,环孢素治疗有效率较低.
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安文彬
张培红
任媛媛
郭晔
王书春
陈晓娟
杨文钰
常丽贤
竺晓凡
关键词 难治性血细胞减少临床特征预后儿童    
Abstract:Objective To study the clinical features of patients with refractory cytopenia of childhood (RCC). Methods The clinical data of 1 420 children (0-14 years old) with an initial diagnosis of non-severe aplastic anemia between January 1990 and June 2013 were retrospectively analyzed. Bone marrow cell morphology and histopathology were re-evaluated, and the patients were re-classified using the criteria proposed in the 2008 edition of the World Health Organization classification of RCC in hematopoietic and lymphoid tumor tissues. The clinical outcomes were followed up every 3-6 months. Results Among all the 1 420 cases, 152 (10.7%) were reassessed as RCC. Patients with RCC had a lower level of hemoglobin and a higher percentage of fetal hemoglobin than those with non-severe aplastic anemia. Of the patients with RCC, 21.5% showed abnormal karyotypes at diagnosis. The median follow-up period for all patients was 36 months (ranging from 1 to 283 months). The rates of complete response, partial response, and no response to cyclosporine and androgen treatment in RCC patients were 19.0%, 26.7%, and 54.3%, respectively. The 5- and 10-year prospective overall survival rates of RCC patients were 87.9% and 72.4%, respectively. The 5- and 10-year prospective clonal evolution rates were 15.3% and 20.0%, respectively. The 2-year prospective incidence of newly diagnosed karyotype abnormality after the initial diagnosis was 3.6%. The 5- and 10-year prospective leukemia transformation rates were 10.0% and 20.0%, respectively. Conclusions RCC shows clinical features similar to adult myelodysplastic syndrome. Children with RCC have a poor prognosis, an increased risk of transformation to leukemia, and a low response rate to cyclosporine treatment.
Key wordsRefractory cytopenia    Clinical feature    Prognosis    Child   
收稿日期: 2014-06-12     
基金资助:2010-2012卫生部部属(管)医院学科重点项目.
通讯作者: 竺晓凡,女,教授.   
作者简介: 安文彬,女,博士,住院医师.
引用本文:   
安文彬,张培红,任媛媛等. 儿童难治性血细胞减少的临床特征分析[J]. 中国当代儿科杂志, 2015, 17(1): 15-21.
AN Wen-Bin,ZHANG Pei-Hong,REN Yuan-Yuan et al. Clinical features of childhood refractory cytopenia[J]. CJCP, 2015, 17(1): 15-21.
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