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巴西亚马逊地区多次输血的镰状细胞病患者迟发性输血反应的调查。

Investigation of Delayed Transfusion Reactions in Sickle Cell Disease Patients Polytransfused in the Brazilian Amazon.

作者信息

Santos Lorena Alves, de Almeida Anne Cristine Gomes, Tarragô Andrea Monteiro, da Silva Nina Rosa Gonçalves, da Silva Juliana Nascimento Vitoriano, de Souza Mônica Moura, Nascimento Monik Oney Oliveira, Nascimento Marcelo Reis do, Castro Ana Caroline Dos Santos, de Albuquerque Cinthia Xerez, Cardoso Evilázio Cunha, Neto José Pereira Moura, Albuquerque Sérgio Roberto Lopes

机构信息

Programa de Pós-Graduação em Ciências Aplicadas à Hematologia da Universidade do Estado do Amazonas (PPGH-UEA), Manaus 69050-001, AM, Brazil.

Programa de Pós-Graduação em Imunologia Básica e Aplicada da Universidade Federal do Amazonas (PPGIBA-UFAM), Manaus 69067-005, AM, Brazil.

出版信息

Hematol Rep. 2024 Aug 1;16(3):512-522. doi: 10.3390/hematolrep16030049.

DOI:10.3390/hematolrep16030049
PMID:39189245
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11348377/
Abstract

BACKGROUND

Sickle cell disease (SCD) affects approximately 100,000 people in the United States and millions worldwide, with the highest prevalence of 70% of SCD being found in individuals of African ethnicity. Delayed hemolytic, alloimmunization, and anamnestic transfusion reactions in multiple transfusion patients need to be investigated and managed to avoid a worsening of the patient's clinical status.

OBJECTIVE

This paper aims to investigate delayed transfusion reactions in SCD patients who were polytransfused in the Brazilian Amazon.

MATERIAL AND METHODS

The clinical and laboratory indicators of SCD patients with more than four transfusions were investigated. The patients were treated at the Fundação Hospitalar de Hematologia e Hemoterapia do Estado do Amazonas, Brazil.

RESULTS

A total of 44 polytransfused patients with SCD were followed. Regarding Rh phenotype, it was possible to observe a frequency of 26.6% (12) patients with the RZRZ (DCE/DCE) phenotype, in addition to 4.5% (two) patients with and variants. It was also possible to observe 20.5% (nine) patients with an alloimmunization reaction, who presented the following alloantibodies: anti-RhD, anti-E, anti-K, anti-Jk, anti-N, anti-S, and anti-Di, two of which are unidentified. Of these, four (44.4%) patients also presented autoantibodies, anti-e, and three unidentified antibodies, and four (44.4%) patients presented an anamnestic reaction, with anti-RhD, K, and Jkb antibodies. Of the 44 patients monitored, 54.4% (24) had clinical and laboratory indicators of a delayed hemolytic reaction.

CONCLUSION

Delayed transfusion reactions, often neglected, occur frequently. Therefore, transfusions need to be monitored for at least 28 days, with medical investigation of clinical and laboratory indicators to make greater use of this therapeutic resource.

摘要

背景

在美国,约有10万人患有镰状细胞病(SCD),全球则有数百万人患病,其中非洲裔个体的SCD患病率最高,达70%。对于多次输血的患者,需要对延迟性溶血、同种免疫和回忆性输血反应进行调查和处理,以避免患者临床状况恶化。

目的

本文旨在调查巴西亚马逊地区接受多次输血的SCD患者的延迟性输血反应。

材料与方法

对接受过四次以上输血的SCD患者的临床和实验室指标进行调查。这些患者在巴西亚马逊州血液学和血液疗法医院接受治疗。

结果

共对44例接受多次输血的SCD患者进行了随访。关于Rh血型表型,可观察到RZRZ(DCE/DCE)表型的患者频率为26.6%(12例),此外还有4.5%(2例)的患者具有其他变体。还可观察到20.5%(9例)的患者发生同种免疫反应,这些患者出现了以下同种抗体:抗-RhD、抗-E、抗-K、抗-Jk、抗-N、抗-S和抗-Di,其中两种未明确。其中,4例(44.4%)患者还出现了自身抗体、抗-e和三种未明确的抗体,4例(44.4%)患者出现了回忆性反应,伴有抗-RhD、K和Jkb抗体。在监测的44例患者中,54.4%(24例)有延迟性溶血反应的临床和实验室指标。

结论

延迟性输血反应经常被忽视,但发生率很高。因此,输血后至少需要监测28天,并对临床和实验室指标进行医学调查,以便更好地利用这种治疗资源。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d29f/11348377/e4aa6a76056e/hematolrep-16-00049-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d29f/11348377/e4aa6a76056e/hematolrep-16-00049-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d29f/11348377/e4aa6a76056e/hematolrep-16-00049-g001.jpg

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本文引用的文献

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Hematol Transfus Cell Ther. 2024 Jul-Sep;46(3):261-267. doi: 10.1016/j.htct.2023.05.004. Epub 2023 Jun 15.
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Red blood cell alloantibodies in paediatric transfusion in sub-Saharan Africa: A new cohort and literature review.撒哈拉以南非洲地区儿科输血中的红细胞同种抗体:一项新的队列研究及文献综述
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Extended phenotyping does not preclude the occurrence of delayed haemolytic transfusion reactions in sickle cell disease.
扩展表型分析并不能排除镰状细胞病迟发性溶血性输血反应的发生。
Br J Haematol. 2022 Feb;196(3):769-776. doi: 10.1111/bjh.17875. Epub 2021 Oct 10.
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The vaso-occlusive pain crisis in sickle cell disease: Definition, pathophysiology, and management.镰状细胞病的血管阻塞性疼痛危象:定义、病理生理学和治疗。
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