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噬血细胞综合征病例报告

A Case Report of Hemophagocytic Syndrome.

作者信息

Mendes Tânia F, Oliveira Ana Isabel, Gomes Carolina, Sousa Nuno A, Gonçalves Pereira João

机构信息

Internal Medicine, Hospital Vila Franca de Xira, Vila Franca de Xira, PRT.

Intensive Care Unit, Hospital Vila Franca de Xira, Vila Franca de Xira, PRT.

出版信息

Cureus. 2025 Feb 17;17(2):e79150. doi: 10.7759/cureus.79150. eCollection 2025 Feb.

DOI:10.7759/cureus.79150
PMID:40109780
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11921754/
Abstract

Hemophagocytic syndrome (HPS) represents a critical and often overlooked hyperinflammatory condition that can lead to rapid multi-organ failure and high mortality rates, particularly in adults. This article presents a compelling case study of a 45-year-old male with a complex clinical presentation, highlighting the diagnostic challenges posed by HPS, including its nonspecific symptoms and the necessity for a high index of suspicion. We underscore the paramount importance of early recognition, thorough differential diagnosis, and prompt initiation of treatment to improve patient outcomes. This case not only illustrates the intricacies of diagnosing HPS but also advocates for increased awareness among healthcare providers to mitigate the risks associated with this life-threatening syndrome.

摘要

噬血细胞综合征(HPS)是一种严重且常被忽视的高炎症状态,可导致快速的多器官功能衰竭和高死亡率,尤其是在成年人中。本文介绍了一例45岁男性的引人注目的病例研究,该病例临床表现复杂,突出了HPS带来的诊断挑战,包括其非特异性症状以及高度怀疑的必要性。我们强调早期识别、全面鉴别诊断和及时开始治疗对改善患者预后的至关重要性。该病例不仅说明了诊断HPS的复杂性,还倡导提高医疗服务提供者的认识,以降低与这种危及生命的综合征相关的风险。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/65ea/11921754/5c6f8413e25b/cureus-0017-00000079150-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/65ea/11921754/fb0bbb89fc9b/cureus-0017-00000079150-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/65ea/11921754/5c6f8413e25b/cureus-0017-00000079150-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/65ea/11921754/fb0bbb89fc9b/cureus-0017-00000079150-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/65ea/11921754/5c6f8413e25b/cureus-0017-00000079150-i06.jpg

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本文引用的文献

1
Delay in treatment of adult hemophagocytic lymphohistiocytosis is associated with worse in-hospital outcomes.成人噬血细胞性淋巴组织细胞增生症治疗延误与住院期间预后不良相关。
Ann Hematol. 2023 Nov;102(11):2989-2996. doi: 10.1007/s00277-023-05271-w. Epub 2023 Jul 1.
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Hemophagocytic lymphohistiocytosis: a review inspired by the COVID-19 pandemic.噬血细胞性淋巴组织细胞增生症:COVID-19 大流行引发的综述。
Rheumatol Int. 2021 Jan;41(1):7-18. doi: 10.1007/s00296-020-04636-y. Epub 2020 Jun 25.
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Adult haemophagocytic lymphohistiocytosis: a Review.
成人噬血细胞性淋巴组织细胞增生症:综述
QJM. 2020 Jan 14. doi: 10.1093/qjmed/hcaa011.
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Hemophagocytic Syndrome-An Approach to the Management.噬血细胞综合征——管理方法
Indian J Crit Care Med. 2019 Sep;23(Suppl 3):S191-S196. doi: 10.5005/jp-journals-10071-23251.
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Recommendations for the management of hemophagocytic lymphohistiocytosis in adults.成人噬血细胞性淋巴组织细胞增生症的治疗建议。
Blood. 2019 Jun 6;133(23):2465-2477. doi: 10.1182/blood.2018894618. Epub 2019 Apr 16.
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Macrophage Activation-Like Syndrome: A Distinct Entity Leading to Early Death in Sepsis.巨噬细胞活化综合征:导致脓毒症早期死亡的独特实体。
Front Immunol. 2019 Jan 31;10:55. doi: 10.3389/fimmu.2019.00055. eCollection 2019.
7
Pathophysiology and epidemiology of hemophagocytic lymphohistiocytosis.噬血细胞性淋巴组织细胞增生症的病理生理学与流行病学
Hematology Am Soc Hematol Educ Program. 2015;2015:177-82. doi: 10.1182/asheducation-2015.1.177.
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Clinical characteristics and prognostic factors of adult hemophagocytic syndrome patients: a retrospective study of increasing awareness of a disease from a single-center in China.成人噬血细胞综合征患者的临床特征及预后因素:一项来自中国单中心对一种疾病认识不断提高的回顾性研究
Orphanet J Rare Dis. 2015 Feb 15;10:20. doi: 10.1186/s13023-015-0224-y.
9
Hemophagocytic lymphohistiocytosis: review of etiologies and management.噬血细胞性淋巴组织细胞增生症:病因及治疗综述
J Blood Med. 2014 Jun 12;5:69-86. doi: 10.2147/JBM.S46255. eCollection 2014.
10
Hemophagocytic lymphohistiocytosis: pathogenesis and treatment.噬血细胞性淋巴组织细胞增生症:发病机制与治疗
Hematology Am Soc Hematol Educ Program. 2013;2013:605-11. doi: 10.1182/asheducation-2013.1.605.