儿童纯红细胞再生障碍性贫血28例回顾分析  

Retrospective analysis of 28 cases of children with pure red cell aplasia

作　　者：姜丽华[1] 陈森[1] 乔丽津[1] 李静岩[1] 刘嫣[1] 

机构地区：[1]天津市儿童医院血液科,天津300070

出　　处：《中国实用儿科杂志》2014年第2期129-132,共4页Chinese Journal of Practical Pediatrics

摘　　要：目的探讨儿童纯红细胞再生障碍性贫血(PRCA)临床特点、治疗及预后。方法对1995年3月至2010年9月天津市儿童医院血液科确诊治疗的28例PRCA患儿进行临床病例资料总结回顾,并对其中15例预后进行跟踪随访。结果1岁以内发病18例(64.3%),男∶女为2.5∶1。12例(42.9%)合并各种先天畸形,以泌尿生殖系统畸形发生率最高(8/28,28.6%)。3例(10.7%)家族史阳性。33.3%(4/12)体液免疫功能紊乱。所有T细胞亚群检测者(9/9)均有CD4+T细胞减少。病原学检测主要为巨细胞病毒(CMV)和微小病毒B19(HPVB19)感染。26例接受肾上腺糖皮质激素治疗,19例接受静脉丙种球蛋白、雄激素及环孢素A联合治疗。跟踪随访的15例中缓解或明显进步10例(66.7%)。结论儿童PRCA尤其Diamond-Blackfan综合征(DBA)发病年龄较小,多在1岁内,常伴多种先天畸形,以泌尿生殖系统畸形最多见。HPVB19及CMV感染可能是诱发儿童获得性PRCA的原因之一。肾上腺糖皮质激素可改善部分PRCA预后,但疗程较长,部分患者可能需终身小剂量维持。合理输血可改善临床症状,提高生存质量。Objective By exploring the clinical features of pure red cell aplastic （PRCA） in children, to improve our knowledge of early diagnosis, therapy methods and prognosis about this disease, and to provide more clinical data of this disease. Methods The single center clinical data of twenty-eight cases of PRCA presented in Tianjin area was reviewed, and 15 cases of them were followed up for clinical prognosis. Results The incidence of patient within 1 year old accounted for 64.3%, and the male to famale ratio was 2.5：1 ;42.9% of the patients can be associated with various congenital malformations, and the most common anomaly was genitourinary malformations （accounting for 28.6% ）. The positive rate of family history was 10.7%. About 33.3% of the patients had humoral immune function disorder, and all of the patients undergoing detection of cellular immunity had reduced CD4＋ cell. Etiological detection revealed mainly CMV and HPVB19 virus infection. Twenty-six patients received eortieosteroid therapy, and 19 received combined immunoglobulin therapy, androgen therapy and eyelosporin A therapy. Fifteen cases of them were followed-up for clinical prognosis and the overall efficiency was 66.7%. Conclusion The onset age of disease is often younger than one-year-old in children with PRCA, especially DBA.PRCA patients often have multiple congenital anomalies, and among them the genitourinary malformations are most common.The male to female ratio of PRCA is higher than that reported in the literature.Positive family history is coincided with the literature. The etiology of PRCA in children is unknown.The HPVB 19 and CMV infection may be the cause of children with acquired PRCA.Corticosteroid can improve the Clinical outcome in part of PRCA patients in children, but it requires a longer course of treatment and some patients may need lifelong small dose maintenance.Blood transfusion could improve the clinical symptoms and improve life quality.

关 键 词：纯红细胞再生障碍性贫血 畸形 肾上腺糖皮质激素 

分 类 号：R556.5[医药卫生—血液循环系统疾病]