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伊文氏综合征的诊断与治疗策略:一例报告及文献综述

Diagnostic and Therapeutic Strategies in Evans Syndrome: A Case Report and Literature Review.

作者信息

Palvia Aadi R, Damera Abhiram Rao, Magar Shikha, Nandi Akshay Rahul, Goyal Mayank

机构信息

Internal Medicine, Kharghar Medicity Hospital, Navi Mumbai, IND.

Internal Medicine, MediCiti Institute of Medical Sciences, Hyderabad, IND.

出版信息

Cureus. 2024 Jul 18;16(7):e64866. doi: 10.7759/cureus.64866. eCollection 2024 Jul.

DOI:10.7759/cureus.64866
PMID:39156320
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11330688/
Abstract

Evans syndrome (ES) is characterized by a combination of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). Immune dysregulation, which results in the development of antibodies against blood cells, is its defining feature. ES being a diagnosis of exclusion requires a thorough workup to rule out other probable illnesses like lymphoproliferative diseases and systemic lupus erythematosus (SLE). We present the case of a 38-year-old male who experienced shortness of breath, chest discomfort, and generalized weakness. His medical history included recurrent anemia, thrombocytopenia, and pulmonary tuberculosis in remission. Hemolysis, thrombocytopenia, and a large pericardial effusion were discovered during the physical examination and investigations. An initial treatment strategy that included pericardiocentesis was performed. In combination with AIHA and ITP, the clinical and laboratory findings strongly suggested ES, which improved with prednisolone therapy. First-line treatments consist of corticosteroids and intravenous immunoglobulin; refractory cases may also require rituximab, thrombopoietin receptor antagonists, and sirolimus. Achieving remission and lowering relapse rates need careful patient monitoring and customized treatment programs.

摘要

伊文斯综合征(ES)的特征是自身免疫性溶血性贫血(AIHA)和免疫性血小板减少症(ITP)同时存在。导致产生抗血细胞抗体的免疫失调是其主要特征。ES作为一种排除性诊断,需要进行全面检查以排除其他可能的疾病,如淋巴增殖性疾病和系统性红斑狼疮(SLE)。我们报告一例38岁男性病例,该患者出现呼吸急促、胸部不适和全身乏力。他的病史包括复发性贫血、血小板减少症以及处于缓解期的肺结核。体格检查和检查发现有溶血、血小板减少和大量心包积液。实施了包括心包穿刺术在内的初始治疗策略。结合AIHA和ITP,临床和实验室检查结果强烈提示为ES,泼尼松龙治疗后病情有所改善。一线治疗包括皮质类固醇和静脉注射免疫球蛋白;难治性病例可能还需要利妥昔单抗、血小板生成素受体拮抗剂和西罗莫司。实现缓解和降低复发率需要对患者进行仔细监测并制定个性化治疗方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c987/11330688/1c7c3ccaef9b/cureus-0016-00000064866-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c987/11330688/84afa0041acd/cureus-0016-00000064866-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c987/11330688/1c7c3ccaef9b/cureus-0016-00000064866-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c987/11330688/84afa0041acd/cureus-0016-00000064866-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c987/11330688/1c7c3ccaef9b/cureus-0016-00000064866-i02.jpg

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本文引用的文献

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Sirolimus is effective for primary refractory/relapsed warm autoimmune haemolytic anaemia/Evans syndrome: a retrospective single-center study.西罗莫司治疗原发性难治/复发性温抗体型自身免疫性溶血性贫血/Evans 综合征有效:一项回顾性单中心研究。
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Blood. 2022 Aug 18;140(7):789-792. doi: 10.1182/blood.2022016818.
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Concurrent severe pulmonary tuberculosis with Evans syndrome: a case report with literature review.
并发严重肺结核和 Evans 综合征:病例报告并文献复习。
BMC Infect Dis. 2022 Jun 13;22(1):533. doi: 10.1186/s12879-022-07512-1.
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JNMA J Nepal Med Assoc. 2022 May 5;60(249):482-484. doi: 10.31729/jnma.7421.
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Primary Evans syndrome in an adult man.成人原发性 Evans 综合征。
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Blood Adv. 2021 Dec 28;5(24):5468-5478. doi: 10.1182/bloodadvances.2021005610.
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Right heart thrombus-in-transit in a patient with Evans syndrome: A case report.患者合并 Evans 综合征发生右心血栓-游走:病例报告。
Medicine (Baltimore). 2021 Aug 20;100(33):e27009. doi: 10.1097/MD.0000000000027009.
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Pan Afr Med J. 2021 Mar 29;38:314. doi: 10.11604/pamj.2021.38.314.22410. eCollection 2021.
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