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6 岁儿童难治性混合自身免疫性溶血性贫血 1 例报告

Rare case of refractory mixed autoimmune hemolytic anemia in a 6-year-old child: a case report.

机构信息

Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada.

Division of Hematopathology, Department of Pediatric Laboratory Medicine, The Hospital for Sick Children, Toronto, ON, Canada.

出版信息

J Med Case Rep. 2023 Oct 5;17(1):418. doi: 10.1186/s13256-023-04154-y.

DOI:10.1186/s13256-023-04154-y
PMID:37794483
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10552242/
Abstract

BACKGROUND

Mixed autoimmune hemolytic anemia (AIHA) shows combined clinical and laboratory characteristics of warm and cold AIHA. It is relatively uncommon in children. Consequently, knowledge about mixed AIHA prevalence, clinical presentation, treatment options, and prognosis in children is limited to very few case reports.

CASE PRESENTATION

We describe a six-year-old Asian girl presenting with profound anemia, blood group typing discrepancy and crossmatch incompatibility, post upper respiratory tract infection. Detection of red cell warm and cold reactive autoantibodies, led to the diagnosis of mixed AIHA. Autoantibodies with laboratory evidence of hemolysis persisted despite high dose steroid therapy. Due to the inability to wean further, the patient was subsequently commenced on mycophenolate mofetil to which she seems to be responding.

CONCLUSIONS

Mixed AIHA may be notoriously difficult to diagnose and treat. Detailed clinical and laboratory work-up is essential to establish the diagnosis. To the best of our knowledge, this is the first case report of mixed AIHA following upper respiratory tract infection. Awareness of this occurrence is important, as similar to warm AIHA, mixed AIHA should be treated immediately by early initiation of steroid therapy. In addition, prompt supportive care as well as long-term clinical follow-up are required to improve outcomes of these cases.

摘要

背景

混合自身免疫性溶血性贫血(AIHA)表现为温抗体型和冷抗体型 AIHA 的联合临床和实验室特征。在儿童中较为少见。因此,关于儿童混合 AIHA 的患病率、临床表现、治疗选择和预后的知识仅限于极少数病例报告。

病例介绍

我们描述了一名六岁的亚洲女孩,在上呼吸道感染后出现严重贫血、血型定型和交叉配血不合、红细胞温冷反应性自身抗体检测,导致混合 AIHA 的诊断。尽管接受了大剂量类固醇治疗,但仍存在具有实验室溶血证据的自身抗体。由于无法进一步停药,随后开始使用吗替麦考酚酯治疗,她似乎对此有反应。

结论

混合 AIHA 可能难以诊断和治疗。详细的临床和实验室检查对于确立诊断至关重要。据我们所知,这是首例上呼吸道感染后发生混合 AIHA 的病例报告。了解这种情况很重要,因为与温抗体型 AIHA 一样,混合 AIHA 也应通过早期开始类固醇治疗立即进行治疗。此外,需要进行及时的支持性护理以及长期的临床随访,以改善这些病例的结局。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea20/10552242/4e04c65f9573/13256_2023_4154_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea20/10552242/4e04c65f9573/13256_2023_4154_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ea20/10552242/4e04c65f9573/13256_2023_4154_Fig1_HTML.jpg

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本文引用的文献

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Autoimmune Hemolytic Anemia in Children: Laboratory Investigation, Disease Associations, and Treatment Strategies.儿童自身免疫性溶血性贫血:实验室检查、疾病关联及治疗策略。
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Autoimmune Hemolytic Anemia in the Pediatric Setting.儿童自身免疫性溶血性贫血
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New Insights in Autoimmune Hemolytic Anemia: From Pathogenesis to Therapy Stage 1.自身免疫性溶血性贫血的新见解:从发病机制到治疗 第一阶段
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