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通过华法林抗凝、静脉注射免疫球蛋白(IVIG)和大剂量皮质类固醇成功治疗Evans综合征中的中风。

The Successful Management of Stroke in Evans Syndrome by Anticoagulation with Warfarin, Intravenous Immunoglobulin (IVIG), and High-Dose Corticosteroid.

作者信息

Takahashi Toshiaki, Takaoka Kensuke, Kwong Kelsey, Macapagal Sharina, Tanariyakul Manasawee, Wannaphut Chalothorn, Nishimura Yoshito

机构信息

Department of Medicine, John A. Burns School of Medicine, University of Hawaii, Honolulu, USA.

出版信息

Eur J Case Rep Intern Med. 2024 Jun 28;11(7):004592. doi: 10.12890/2024_004592. eCollection 2024.

Abstract

UNLABELLED

Evans syndrome (ES) is a rare autoimmune disorder characterised by autoimmune haemolytic anaemia (AIHA), immune thrombocytopenia and autoimmune neutropenia. The precise pathogenesis of ES remains unclear, but it is believed to involve immune-mediated destruction of erythrocytes and platelets. Thrombotic complications, such as stroke, are critical yet largely unrecognised in ES. Here, we present a case of an 80-year-old male with ES who developed multiple strokes, emphasising the complex management challenges associated with this condition. The patient, known for stage IIB lung adenocarcinoma, presented with right-sided weakness and was diagnosed with a stroke of undetermined aetiology. He was started on warfarin for secondary prevention alongside intravenous immunoglobulin (IVIG) and corticosteroids for ES. Stroke in ES is rarely reported, and the optimal management remains inconclusive due to its rarity. The patient's management was guided by existing guidelines for stroke prevention and anticoagulation in the setting of antiphospholipid syndrome. While anticoagulants are recommended for venous thromboembolism prophylaxis in AIHA, there are no clear guidelines for stroke prevention in ES. This case underscores the necessity of individualised treatment approaches and highlights the gaps in evidence regarding stroke management in ES. Future research is essential to determine the optimal management of stroke in this complex clinical scenario.

LEARNING POINTS

Evans syndrome is a rare autoimmune disorder characterised by the coexistence of autoimmune haemolytic anaemia and immune thrombocytopenia, which potentially increase venous and arterial thrombotic risk.Managing strokes in Evans syndrome remains challenging due to its rarity and lack of definitive guidelines, necessitating individualised treatment approaches.Future prospective studies are warranted to determine the optimal patient population that needs secondary prevention with anticoagulants following a stroke in the context of Evans syndrome.

摘要

未标注

伊文氏综合征(ES)是一种罕见的自身免疫性疾病,其特征为自身免疫性溶血性贫血(AIHA)、免疫性血小板减少症和自身免疫性中性粒细胞减少症。ES的确切发病机制尚不清楚,但据信涉及免疫介导的红细胞和血小板破坏。血栓形成并发症,如中风,在ES中很关键但在很大程度上未被认识到。在此,我们报告一例80岁男性ES患者发生多次中风的病例,强调了与该疾病相关的复杂管理挑战。该患者以IIB期肺腺癌而闻名,出现右侧肢体无力,被诊断为病因不明的中风。他开始服用华法林进行二级预防,同时使用静脉注射免疫球蛋白(IVIG)和皮质类固醇治疗ES。ES中的中风很少被报道,由于其罕见性,最佳管理仍无定论。患者的管理遵循抗磷脂综合征背景下中风预防和抗凝的现有指南。虽然推荐使用抗凝剂预防AIHA中的静脉血栓栓塞,但ES中中风预防尚无明确指南。该病例强调了个体化治疗方法的必要性,并突出了ES中风管理方面的证据空白。未来的研究对于确定这种复杂临床情况下中风的最佳管理至关重要。

学习要点

伊文氏综合征是一种罕见的自身免疫性疾病,其特征为自身免疫性溶血性贫血和免疫性血小板减少症并存,这可能增加静脉和动脉血栓形成风险。由于其罕见性和缺乏明确指南,管理伊文氏综合征中的中风仍然具有挑战性,需要个体化治疗方法。未来有必要进行前瞻性研究,以确定在伊文氏综合征背景下中风后需要抗凝二级预防的最佳患者群体。

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