16例儿童单纯红细胞再生障碍性贫血临床分析  

Pure red cell aplasia in children: a clinical analysis of 16 cases

作　　者：王忠健 邱奕宁 余慧[1] 谈玲芳[1] 瞿萍 金润铭[1] WANG Zhong-Jian;QIU Yi-Ning;YU Hui;TAN Ling-Fang;QU Ping;JIN Run-Ming(Department of Pediatrics,Union Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430022,China)

机构地区：[1]华中科技大学同济医学院附属协和医院儿科

出　　处：《中国当代儿科杂志》2019年第8期772-776,共5页Chinese Journal of Contemporary Pediatrics

摘　　要：目的探讨儿童单纯红细胞再生障碍性贫血(PRCA)的临床特点、治疗及预后。方法回顾性分析16例PRCA患儿的临床资料,并对比分析泼尼松联合槐杞黄颗粒与单纯泼尼松治疗PRCA的疗效及预后等情况。结果16例患儿均以面色苍白、面色苍黄等贫血症状为主诉。12例进行了病原体检查,7例(58%)存在病原体感染,以人巨细胞病毒最常见。7例行淋巴细胞亚群检查,5例(71%)存在淋巴细胞免疫紊乱。6例患儿行免疫球蛋白及补体检查,均存在异常。泼尼松联合槐杞黄颗粒治疗组(n=8)的中位随访时间为21.5个月,基本治愈1例,缓解1例,明显进步6例,中位起效时间1个月,药物减量或停药过程中出现复发2例。单纯泼尼松治疗组(n=8)的中位随访时间为34个月,基本治愈4例,缓解0例,明显进步4例,中位起效时间为2.5个月,药物减量或停药过程中出现复发4例。结论PRCA患儿一般以贫血相关症状为主诉就诊;部分患儿存在病原体感染,多数患儿伴有机体免疫功能紊乱。糖皮质激素治疗效果较好,但部分患儿在药物减量或停药过程中复发,其中泼尼松联合槐杞黄颗粒治疗起效可能更快、复发可能性更小。Objective To study the clinical features,treatment,and prognosis of pure red cell aplasia(PRCA)in children.Methods A retrospective analysis was performed for the clinical data of 16 children with PRCA.The outcome and prognosis of patients treated with prednisone combined with Huaiqihuang granules versus prednisone alone were evaluated.Results All the 16 children complained of symptoms of anemia including pale or sallow complexion.Of 12 children undergoing pathogen test,7(58%)were found to have pathogen infection,among which human cytomegalovirus was the most common.Lymphocyte subsets were measured for 7 children,among whom 5(71%)had lymphocyte immune disorder.Six children were found to have abnormalities in immunoglobulin and complement.The 8 children treated with prednisone combined with Huaiqihuang granules had a median follow-up time of 21.5 months,among whom 1 was almost cured,1 was relieved,and 6 were obviously improved;the median onset time of treatment was 1 month,and 2 children had disease recurrence in the course of drug reduction or withdrawal.The 8 children in the prednisone alone treatment group had a median follow-up time of 34 months,among whom 4 were almost cured,and 4 were obviously improved;the median onset time of treatment was 2.5 months,and 4 children had recurrence during drug reduction or withdrawal.Conclusions Children with PRCA usually complain of anemia-related symptoms.Laboratory tests show pathogen infection in some children with PRCA,and most of children have immune disorders.Glucocorticoids have a good therapeutic effect,but some children relapse in the course of drug reduction or withdrawal.Combined treatment with prednisone and Huaiqihuang granules may have a faster onset of action and less possibility of recurrence.

关 键 词：单纯红细胞再生障碍性贫血 临床特点 槐杞黄颗粒 治疗 预后 儿童 

分 类 号：R55[医药卫生—血液循环系统疾病]