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儿童自身免疫性溶血性贫血:100例血清学特征

Autoimmune hemolytic anemia in childhood: serologic features in 100 cases.

作者信息

Vaglio Stefania, Arista Maria Cristina, Perrone Maria Paola, Tomei Gabriella, Testi Anna Maria, Coluzzi Serelina, Girelli Gabriella

机构信息

Blood Bank, "La Sapienza" University-II Faculty of Medicine, Rome, Italy.

出版信息

Transfusion. 2007 Jan;47(1):50-4. doi: 10.1111/j.1537-2995.2007.01062.x.

Abstract

BACKGROUND

Red blood cell (RBC) autoimmunization is a relatively uncommon cause of anemia in children and presents some differences from those of adults. Due to its frequency, autoimmune hemolytic anemia (AIHA) in childhood has prompted very few studies, and the literature consists mostly of sporadic case histories. The objective of this study was to stress the importance of an appropriate serologic diagnosis in suspected cases.

STUDY DESIGN AND METHODS

This report describes the immunohematologic features of 100 patients with AIHA studied in the Immunohaematologic Unit of Blood Bank, "La Sapienza" University of Rome. The patients were diagnosed in the same department from 1983 to 2003.

RESULTS

The peak incidence of AIHA was in the first 4 years of life. No sex predominance was noted. Warm AIHA was the most common type of acquired immune hemolytic anemia; it comprised 64 of the 100 patients, whereas 26 patients showed a cold AIHA. Associated AIHA showed a slightly more frequent incidence (54/100) compared to idiopathic forms of AIHA (46/100).

CONCLUSIONS

In this study serologic records of 100 children with confirmed AIHA are reported. This series, much larger than any previously reported, is critically reviewed and analyzed to delineate the immunologic features of the disease in childhood.

摘要

背景

红细胞自身免疫是儿童贫血相对少见的病因,与成人贫血存在一些差异。由于其发病率较低,儿童自身免疫性溶血性贫血(AIHA)的相关研究很少,文献大多为散发病例报告。本研究的目的是强调在疑似病例中进行恰当血清学诊断的重要性。

研究设计与方法

本报告描述了罗马“La Sapienza”大学血库免疫血液学科室研究的100例AIHA患者的免疫血液学特征。这些患者于1983年至2003年在同一科室确诊。

结果

AIHA的发病高峰在生命的前4年。未发现性别优势。温抗体型AIHA是获得性免疫性溶血性贫血最常见的类型;100例患者中有64例为此类型,而26例表现为冷抗体型AIHA。与特发性AIHA(46/100)相比,伴发AIHA的发病率略高(54/100)。

结论

本研究报告了100例确诊AIHA儿童的血清学记录。该系列病例数量远多于以往任何报告,对其进行了严格审查和分析,以明确儿童期该疾病的免疫学特征。

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