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儿童慢性中性粒细胞减少症:来自单一中心的经验

Chronic Neutropenia in Childhood: Experience From a Single Center.

作者信息

Karapinar Tuba H, Oymak Yeşim, Ay Yilmaz, Köker Sultan A, Töret Ersin, Hazan Filiz, Vergin Canan

机构信息

Departments of *Pediatric Hematology and Oncology †Genetics, Dr Behçet Uz Children Research and Training Hospital, Izmir, Turkey.

出版信息

J Pediatr Hematol Oncol. 2016 Jan;38(1):e35-8. doi: 10.1097/MPH.0000000000000449.

Abstract

Chronic neutropenia (CN) is defined as neutropenia that persists for >3 months; it is caused by a heterogeneous group of diseases in children. The aim of the present study was to evaluate the significance and clinical manifestations of CN in children at a single children's hospital. Between October 2004 and April 2014, CN patient data were evaluated retrospectively. Thirty-one patients were assessed in this study. Thirteen of them (41.9%) were younger than 12 months of age at initial diagnosis. There was no difference in the absolute number of neutrophils at diagnosis between the children aged younger than 12 months and those aged 12 months and older at CN onset. Twenty-two of the patients (70.9%) were diagnosed during treatment for acute infections. A total of 11 patients (35.5%) were hospitalized because of recurrent infections. Most of the recurrent infections occurred in the lungs (81.8%). Congenital neutropenia (CoN) was identified in 14 patients (45.1%). Eight of 14 patients (57.1%) required granulocyte-colony stimulating factor treatment, and none of them experienced adverse effects from this treatment. Fifteen patients (48.3%) were diagnosed with idiopathic neutropenia. Comparison between the idiopathic and CoN groups revealed no differences in age, the absolute number of neutrophils, or the presence of infection at diagnosis; however, differences were detected in sex and the rate of spontaneous recovery from neutropenia. Ten of the patients (32.2%) experienced spontaneous recovery from neutropenia during a follow-up period of 7 to 52 months. In current study, we found a higher CoN ratio in the CN patients compared with previous reports, which may be due to the high rate of consanguineous marriages in our country. However, the finding of CN requires several laboratory investigations, prolonged follow-up, and advanced molecular analysis, and its etiology can remain idiopathic.

摘要

慢性中性粒细胞减少症(CN)被定义为持续超过3个月的中性粒细胞减少症;它是由儿童中一组异质性疾病引起的。本研究的目的是评估一家儿童医院中儿童CN的意义和临床表现。2004年10月至2014年4月期间,对CN患者数据进行了回顾性评估。本研究评估了31例患者。其中13例(41.9%)在初次诊断时年龄小于12个月。CN发病时年龄小于12个月的儿童与年龄在12个月及以上的儿童在诊断时中性粒细胞的绝对数量上没有差异。22例患者(70.9%)在急性感染治疗期间被诊断出。共有11例患者(35.5%)因反复感染住院。大多数反复感染发生在肺部(81.8%)。14例患者(45.1%)被诊断为先天性中性粒细胞减少症(CoN)。14例患者中有8例(57.1%)需要粒细胞集落刺激因子治疗,且无一例出现该治疗的不良反应。15例患者(48.3%)被诊断为特发性中性粒细胞减少症。特发性组与CoN组在年龄、中性粒细胞绝对数量或诊断时是否存在感染方面无差异;然而,在性别和中性粒细胞减少症的自发恢复率方面存在差异。10例患者(

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