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儿童免疫性中性粒细胞减少症临床特征研究

孙青，谢瑶，吴鹏辉，李硕，赵卫红 * 扫描二维码
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【摘要】 背景 免疫性中性粒细胞减少症（AIN）是儿童慢性中性粒细胞减少症最常见的类型，但目前国内外
鲜有关于儿童 AIN 的报道。目的 探讨 AIN 患儿的诊治、临床特征和随访资料，为临床医师提供参考。方法 回顾性
收集 2012—2020 年在北京大学第一医院儿科治疗的 22 例 AIN 患儿的临床资料，分析其临床特征及随访情况。结果
22 例 AIN 患儿中男 15 例，女 7 例；初诊中位年龄为 6 个月。共收集血常规检测结果 82 例次，51 例次（62.2%）达到
重度中性粒细胞减少，25 例次（30.5%）为中度中性粒细胞减少，6 例次（7.3%）为轻度中性粒细胞减少。有 2 例患
儿（9.1%）外周血中性粒细胞绝对值计数（ANC）最低值达中度中性粒细胞减少，有 20 例患儿（90.1%）外周血 ANC
最低值达重度中性粒细胞减少。6 例患儿（27.3%）除中性粒细胞减少外，还同时合并其他的临床表现；12 例患儿（54.5%）
完善骨髓细胞形态学检查；6 例患儿（27.3%）完善基因检测。感染发生率为 95.5%，严重感染发生率为 54.5%，没有
患儿发生危及生命的感染；最常见的感染源是病毒，最常见的感染部位是上呼吸道。截至 2021 年 9 月，共有 11 例患
儿外周血 ANC 恢复正常，中位病程为 25（7~42）个月。结论 多数 AIN 患儿外周血 ANC 呈中至重度减少，这类患儿
容易合并感染，最常见的感染源是病毒，最常见感染部位是上呼吸道，但极少发生危及生命的感染。AIN 是自限性疾病，
本报道 AIN 患儿的中位病程为 25 个月。
【关键词】 中性粒细胞减少；儿童；自身抗体；血液和免疫系统；感染
【中图分类号】 R 557.1 【文献标识码】 A DOI：10.12114/j.issn.1007-9572.2021.01.605
孙青，谢瑶，吴鹏辉，等 . 儿童免疫性中性粒细胞减少症临床特征研究［J］. 中国全科医学，2022，25（8）：
969-972，978. ［www.chinagp.net］
SUN Q，XIE Y，WU P H，et al. Autoimmune neutropenia in children：clinical characteristics［J］. Chinese General
Practice，2022，25（8）：969-972，978.

Autoimmune Neutropenia in Children：Clinical Characteristics SUN Qing，XIE Yao，WU Penghui，LI Shuo，ZHAO
Weihong *
Department of Pediatrics，Peking University First Hospital，Beijing 100034，China
*
Corresponding author：ZHAO Weihong，Chief physician；E-mail：zhaowh3212@126.com
【Abstract】 Background Autoimmune neutropenia （AIN） is a common chronic neutropenia in children，but it in children
has been rarely reported. Objective To analyse clinical characteristics，diagnosis，treatment，and follow-up of children with AIN，
aiming at providing support for clinicians. Methods A retrospective design was used. Participants were 22 children with AIN recruited
from Department of Pediatrics，Peking University First Hospital from 2012 to 2020. Their clinical and follow-up data were collected and
analyzed. Results Of the 22 cases，15 were male and 7 were female；with 6 months as the median initial diagnosis age. Among the
results of 82 routine blood tests performed for all cases，severe，moderate and mild neutropenia accounted for 62.2%（51/82），30.5%
（25/82），and 7.3%（6/82），respectively. Two cases（9.1%） were diagnosed with moderate neutropenia and other 20（90.1%）
were diagnosed with severe neutropenia by the lowest absolute neutrophil count（ANC）. Six children （27.3%） also had other clinical
manifestations besides neutropenia. Twelve cases（54.5%） underwent the examination of bone marrow cell morphologies. Six cases （27.3%）
underwent gene test. 95.5% of the cases had infections，and 54.5% of them had severe infections，but no life-threatening infections. The
leading pathogen of infections was virus. The upper respiratory tract was the most common infected area. By the end of September 2021，
the ANC of 11 cases returned to the normal level，and the median of AIN of them was 25 （range: 7-42） months. Conclusion Most
AIN children had moderate to severe neutropenia，and they were prone to infections mostly caused by virus，and the most usually were
upper respiratory tract infections，but rarely life-threatening. The median duration of AIN，a self-limited disease，for our reported cases
was 25 months.
【Key words】 Neutropenia；Child；Autoantibodies；Hemic and immune systems；Infections

100034 北京市，北京大学第一医院儿科
*
通信作者：赵卫红，主任医师；E-mail：zhaowh3212@126.com
本文数字出版日期：2022-01-28