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儿童一过性红细胞生成减少症是一种诊断不足的自限性疾病。

Transient erythroblastopenia of childhood is an underdiagnosed and self-limiting disease.

作者信息

van den Akker Machiel, Dror Yigal, Odame Isaac

机构信息

Division of Haematology/Oncology, Hospital for Sick Children, Toronto, ON, Canada.

出版信息

Acta Paediatr. 2014 Jul;103(7):e288-94. doi: 10.1111/apa.12634. Epub 2014 Apr 29.

DOI:10.1111/apa.12634
PMID:24635829
Abstract

AIM

Transient erythroblastopenia of childhood (TEC) is an uncommon, benign normocytic anaemia of unknown cause, characterised by reduced or absent mature erythroid precursors in otherwise normocellular bone marrow and a complete spontaneous recovery. We present epidemiological data on paediatric TEC cases in a single centre over 30 years and compare them with published data.

METHODS

In this retrospective study, epidemiological data on children diagnosed with TEC between 1978 and 2008 were collected and compared with published data.

RESULTS

A total of 36 children (median age 19 months, 56% male children) were diagnosed. At presentation, median haemoglobin was 44 g/L with absolute reticulocyte count 0 × 10(9) /L; seventeen (47%) patients were neutropenic and 23 (64%) had platelet counts of more than 400 × 10(9) /L. The majority (78%) presented from 1983 to 1997, and 78% of articles reviewing 10 or more TEC patients were published between 1983 and 1992.

CONCLUSION

Transient erythroblastopenia of childhood is now diagnosed less frequently in our institution than in the last two decades. Although the aetiology remains largely unknown, it is possible that changes in causative environmental factors contribute to making TEC a rare disease. Clinicians need to be aware of TEC in order to prevent unnecessary diagnostic and therapeutic measurements.

摘要

目的

儿童短暂性红细胞生成减少症(TEC)是一种罕见的、病因不明的良性正细胞性贫血,其特征是在正常细胞的骨髓中成熟红细胞前体减少或缺失,并能完全自发恢复。我们呈现了一个单一中心30年来儿科TEC病例的流行病学数据,并将其与已发表的数据进行比较。

方法

在这项回顾性研究中,收集了1978年至2008年间诊断为TEC的儿童的流行病学数据,并与已发表的数据进行比较。

结果

共诊断出36名儿童(中位年龄19个月,56%为男性儿童)。就诊时,中位血红蛋白为44g/L,绝对网织红细胞计数为0×10⁹/L;17名(47%)患者有中性粒细胞减少,23名(64%)患者血小板计数超过400×10⁹/L。大多数(78%)病例出现在1983年至1997年,且78%的回顾10例或更多TEC患者的文章发表于1983年至1992年之间。

结论

与过去二十年相比,目前我们机构诊断出的儿童短暂性红细胞生成减少症较少。尽管病因在很大程度上仍不清楚,但致病环境因素的变化有可能导致TEC成为一种罕见疾病。临床医生需要了解TEC,以避免不必要的诊断和治疗措施。

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1
Transient erythroblastopenia of childhood is an underdiagnosed and self-limiting disease.儿童一过性红细胞生成减少症是一种诊断不足的自限性疾病。
Acta Paediatr. 2014 Jul;103(7):e288-94. doi: 10.1111/apa.12634. Epub 2014 Apr 29.
2
[Transient erythroblastopenia in children; a harmless form of anemia].[儿童短暂性成红细胞减少症;一种无害的贫血形式]
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[Transient erythroblastopenia in children. Severe anemia with good prognosis].[儿童短暂性成红细胞减少症。预后良好的严重贫血]
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Breath-holding spells occur disproportionately more often in children with transient erythroblastopenia.屏气发作在患有暂时性红细胞生成减少症的儿童中发生的比例尤其更高。
Acta Paediatr. 2016 Sep;105(9):1088-93. doi: 10.1111/apa.13428. Epub 2016 Apr 29.
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[Transient erythroblastopenia in childhood].[儿童期短暂性红细胞生成减少症]
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Fetal-like erythropoiesis during recovery from transient erythroblastopenia of childhood (TEC).儿童暂时性红细胞生成减少症(TEC)恢复期间的胎儿样红细胞生成。
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Diagnosis and management of red cell aplasia in children.儿童红细胞再生障碍的诊断与管理
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[Severe anemia caused by transient erythroblastopenia in young children].[幼儿短暂性成红细胞减少症所致严重贫血]
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Transient erythroblastopenia of childhood (TEC) presenting as leukoerythroblastic anemia.表现为幼稚粒-幼红细胞性贫血的儿童一过性红细胞生成减少症(TEC)。
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