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[儿童红细胞生成减少症]

[Erythroblastopenia in children].

作者信息

Coulombel L

出版信息

Rev Prat. 1989 Oct 21;39(24):2138-42.

PMID:2554485
Abstract

The occurrence of isolated erythroblastopenia is a common problem in paediatrics, and in most cases three diagnoses may be considered: Blackfan-Diamond anaemia (congenital erythroblastopenia), transient erythroblastopenia of childhood and erythroblastopenia consecutive to parvovirus B19 infection. These three diseases have distinctive features: age of onset, association with other laboratory and clinical abnormalities and above all, transient or chronic character are of assistance in making an exact diagnosis. The mechanisms responsible for Blackfan-Diamond anaemia and transient erythroblastopenia are imperfectly known. In vitro studies of the properties of erythroblast progenitors suggest intrinsic damage to these cells, whereas in transient erythroblastopenia their differentiation seems to be inhibited, perhaps by an immune mechanism. In both cases, the abnormality has not be identified. The selective tropism of parvovirus B19 towards actively dividing erythroblasts accounts for the acute erythroblastopenia observed in a context of chronic haemolysis. The responsibility of parvovirus B19 in chronic or relapsing erythroblastopenia in immunocompromised patients is a recent discovery. In view of the protean haematological pathology in this context and of the difficult therapeutic problems it creates, this diagnosis must systematically be envisaged, notably in children under chemotherapy.

摘要

单纯红细胞生成减少症在儿科是一个常见问题,在大多数情况下可考虑三种诊断:先天性纯红细胞再生障碍性贫血(即黑-戴二氏贫血,先天性红细胞生成减少症)、儿童期短暂性红细胞生成减少症以及细小病毒B19感染后继发性红细胞生成减少症。这三种疾病具有不同特征:发病年龄、与其他实验室及临床异常情况的关联,最重要的是,其短暂性或慢性特征有助于做出准确诊断。导致黑-戴二氏贫血和儿童期短暂性红细胞生成减少症的机制尚不完全清楚。对成红细胞祖细胞特性的体外研究表明这些细胞存在内在损伤,而在儿童期短暂性红细胞生成减少症中,其分化似乎受到抑制,可能是通过免疫机制。在这两种情况下,异常情况均未得到明确。细小病毒B19对活跃分裂的成红细胞具有选择性嗜性,这解释了在慢性溶血背景下观察到的急性红细胞生成减少症。细小病毒B19在免疫功能低下患者的慢性或复发性红细胞生成减少症中的作用是最近才发现的。鉴于这种情况下血液学病理表现多样以及由此产生的治疗难题,必须系统地考虑这一诊断,尤其是在接受化疗的儿童中。

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