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儿童中性粒细胞减少症——叙述性综述与实用诊断方法

Neutropenia in Childhood-A Narrative Review and Practical Diagnostic Approach.

作者信息

Katsaras Georgios, Koutsi Silouani, Psaroulaki Evdokia, Gouni Dimitra, Tsitsani Pelagia

机构信息

Paediatric Department, General Hospital of Pella-Hospital Unit of Edessa, 58200 Edessa, Greece.

Paediatric Outpatient Department, Health Care Center of Aridaia, 58400 Aridaia, Greece.

出版信息

Hematol Rep. 2024 Jun 16;16(2):375-389. doi: 10.3390/hematolrep16020038.

DOI:10.3390/hematolrep16020038
PMID:38921186
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11203312/
Abstract

Neutropenia refers to a decrease in the absolute neutrophil count according to age and race norms and poses a common concern in pediatric practice. Neutrophils serve as host defenders and act crucially in acute inflammation procedures. In this narrative review, we systematically present causes of neutropenia in childhood, mainly adopting the pathophysiological classification of Frater, thereby studying (1) neutropenia with reduced bone marrow reserve, (2) secondary neutropenia with reduced bone marrow reserve, and (3) neutropenia with normal bone marrow reserve. Different conditions in each category are thoroughly discussed and practically approached from the clinician's point of view. Secondary mild to moderate neutropenia is usually benign due to childhood viral infections and is expected to resolve in 2-4 weeks. Bacterial and fungal agents are also associated with transient neutropenia, although fever with severe neutropenia constitutes a medical emergency. Drug-induced and immune neutropenias should be suspected following a careful history and a detailed clinical examination. Cytotoxic chemotherapies treating malignancies are responsible for severe neutropenia and neutropenic shock. Rare genetic neutropenias usually manifest with major infections early in life. Our review of taxonomies clinical findings and associates them to specific neutropenia disorders. We consequently propose a practical diagnostic algorithm for managing neutropenic children.

摘要

中性粒细胞减少症是指根据年龄和种族标准,绝对中性粒细胞计数降低,这在儿科临床实践中是一个常见问题。中性粒细胞作为宿主防御细胞,在急性炎症过程中发挥关键作用。在这篇叙述性综述中,我们系统地介绍了儿童中性粒细胞减少症的病因,主要采用弗拉特的病理生理分类方法,从而研究(1)骨髓储备减少的中性粒细胞减少症,(2)骨髓储备减少的继发性中性粒细胞减少症,以及(3)骨髓储备正常的中性粒细胞减少症。从临床医生的角度,对每一类中的不同情况进行了深入讨论并给出了实际处理方法。继发性轻至中度中性粒细胞减少症通常因儿童病毒感染而呈良性,预计在2 - 4周内缓解。细菌和真菌病原体也与短暂性中性粒细胞减少症有关,尽管严重中性粒细胞减少症伴发热构成医疗急症。在详细询问病史和进行全面临床检查后,应怀疑药物性和免疫性中性粒细胞减少症。治疗恶性肿瘤的细胞毒性化疗会导致严重中性粒细胞减少症和中性粒细胞减少性休克。罕见的遗传性中性粒细胞减少症通常在生命早期就表现为严重感染。我们对分类法、临床发现进行了综述,并将它们与特定的中性粒细胞减少症疾病相关联。因此,我们提出了一种用于管理中性粒细胞减少症儿童的实用诊断算法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1fc/11203312/dd81bce31840/hematolrep-16-00038-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1fc/11203312/dd81bce31840/hematolrep-16-00038-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1fc/11203312/dd81bce31840/hematolrep-16-00038-g001.jpg

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J Clin Pathol. 2024 Aug 16;77(9):586-604. doi: 10.1136/jcp-2022-208686.
2
Dyskeratosis congenita: natural history of the disease through the study of a cohort of patients diagnosed in childhood.先天性角化不良:通过对一组童年期确诊患者的研究了解该疾病的自然史。
Front Pediatr. 2023 Aug 1;11:1182476. doi: 10.3389/fped.2023.1182476. eCollection 2023.
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Autoimmune Neutropenias: Update on Clinical and Biological Features in Children and Adults.
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Hemasphere. 2022 Dec 19;7(1):e814. doi: 10.1097/HS9.0000000000000814. eCollection 2023 Jan.
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Severe congenital neutropenia caused by ELANE gene mutation: A case report and literature review.严重先天性中性粒细胞减少症由 ELANE 基因突变引起:病例报告及文献复习。
Medicine (Baltimore). 2022 Nov 4;101(44):e31357. doi: 10.1097/MD.0000000000031357.
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Etiological causes and prognosis in children with neutropenia.儿童中性粒细胞减少症的病因及预后
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