儿童再生障碍性贫血:目前的鉴别诊断概念

O. Goronkova, A. Pavlova, E. Raykina
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引用次数: 0

摘要

本文简要概述了有关小儿再生障碍性贫血(AA)及密切相关疾病的出版物。在此,我们探讨了再生障碍性贫血的病理生理学,包括骨髓破坏导致再生障碍的三种主要机制:直接损伤、免疫介导的破坏以及遗传性和克隆性疾病导致的骨髓衰竭。遗传性骨髓衰竭综合征、先天性免疫错误和骨髓增生异常综合征是儿童AA鉴别诊断中最常见的疾病。文中介绍了诊断儿童 AA 的综合算法,包括标准实验室检测和其他现代分子与遗传技术,这些技术有助于更好地了解这组异质性疾病,并确定治疗方法的选择。这篇综述的目的是在分析最新文献数据的基础上,为儿科医生和儿科血液病专家提供有关这种罕见、异质性疾病的最新信息。
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Aplastic anemia in children: the current concept of differential diagnosis
This article presents a brief overview of publications on pediatric aplastic anemia (AA) and closely related conditions. Here we consider the pathophysiology of AA, which includes three main mechanisms of bone marrow destruction resulting in aplasia: direct injury, immune mediated destruction and bone marrow failure resulting from inherited and clonal disorders. New aspects of inherited bone marrow failure syndromes, inborn errors of immunity and myelodysplastic syndromes are highlighted as the most common conditions included in the spectrum of differential diagnosis of AA in children. A comprehensive algorithm for the diagnosis of AA in children is presented, including standard laboratory tests and additional modern molecular and genetic techniques that contribute to a better understanding of this heterogeneous group of diseases and determine approaches to the choice of therapy. The purpose of the review is to provide pediatricians and pediatric hematologists with an updated information of this rare, heterogeneous condition based on an analysis of the latest literature data.
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来源期刊
Pediatric Hematology/Oncology and Immunopathology
Pediatric Hematology/Oncology and Immunopathology Medicine-Pediatrics, Perinatology and Child Health
CiteScore
0.40
自引率
0.00%
发文量
49
期刊最新文献
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