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儿童患者直接抗球蛋白试验阴性自身免疫性溶血性贫血病例系列。

A case series of pediatric patients with direct antiglobulin test negative autoimmune hemolytic anemia.

机构信息

Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee.

Department of Pathology, Division of Transfusion Medicine, Stanford University School of Medicine, Stanford, California.

出版信息

Transfusion. 2019 Aug;59(8):2528-2531. doi: 10.1111/trf.15350. Epub 2019 May 21.

Abstract

BACKGROUND

The diagnosis of autoimmune hemolytic anemia (AIHA) can be challenging since the direct antiglobulin test (DAT) has been reported to be falsely negative in 3%-11% of cases. In children with anemia, laboratory and/or clinical evidence of hemolysis and a negative DAT, clinicians should consider further specialized testing to confirm AIHA to accurately diagnose and treat this uncommon pediatric entity.

STUDY DESIGN AND METHODS

A retrospective chart review was undertaken at a large tertiary care academic pediatric hematology practice to describe our experience with DAT-negative AIHA.

RESULTS

From January 1, 2010 through August 1, 2016, 10 children were described who had clinical and laboratory evidence of AIHA, a negative DAT, and further specialized serologic testing confirming this diagnosis.

CONCLUSION

This case series highlights the need for further serologic workup when a child's clinical presentation is highly consistent with AIHA despite a negative DAT.

摘要

背景

自身免疫性溶血性贫血 (AIHA) 的诊断具有一定挑战性,因为已有报道称 3%-11%的病例直接抗球蛋白试验 (DAT) 结果呈假阴性。对于贫血儿童,如果实验室和/或临床有溶血的证据且 DAT 为阴性,临床医生应考虑进一步进行专门的检测以明确 AIHA 的诊断,从而准确诊断和治疗这种罕见的儿科疾病。

研究设计和方法

在一家大型三级儿童血液学学术医疗中心进行了回顾性图表审查,以描述我们在 DAT 阴性 AIHA 方面的经验。

结果

2010 年 1 月 1 日至 2016 年 8 月 1 日,共描述了 10 名患儿,他们均具有 AIHA 的临床和实验室证据、DAT 阴性和进一步的专门血清学检测以确认该诊断。

结论

尽管 DAT 为阴性,但当患儿的临床表现高度符合 AIHA 时,本病例系列强调了需要进行进一步的血清学检查。

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