通过华法林、静脉注射免疫球蛋白 (IVIG) 和高剂量皮质类固醇抗凝成功治疗埃文斯综合征中风。
The Successful Management of Stroke in Evans Syndrome by Anticoagulation with Warfarin, Intravenous Immunoglobulin (IVIG), and High-Dose Corticosteroid.
发表日期:2024
作者:
Toshiaki Takahashi, Kensuke Takaoka, Kelsey Kwong, Sharina Macapagal, Manasawee Tanariyakul, Chalothorn Wannaphut, Yoshito Nishimura
来源:
Immunity & Ageing
摘要:
埃文斯综合征(ES)是一种罕见的自身免疫性疾病,其特征是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。血栓并发症,如中风,在 ES 中很重要,但在很大程度上未被认识到。在这里,我们介绍了一名患有 ES 的 80 岁男性,他出现多次中风,强调了与这种情况相关的复杂的管理挑战。该患者患有 IIB 期肺腺癌,表现为右侧无力,并被诊断为病因不明的中风。他开始使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 中的卒中很少有报道,并且由于其罕见,最佳治疗方法仍无定论。该患者的治疗遵循抗磷脂综合征背景下卒中预防和抗凝的现有指南。虽然推荐使用抗凝剂来预防 AIHA 的静脉血栓栓塞,但对于 ES 的卒中预防尚无明确的指南。该病例强调了个体化治疗方法的必要性,并强调了关于 ES 卒中管理的证据差距。未来的研究对于确定这种复杂临床情况下中风的最佳治疗至关重要。埃文斯综合征是一种罕见的自身免疫性疾病,其特征是自身免疫性溶血性贫血和免疫性血小板减少症并存,这可能会增加静脉和动脉血栓形成的风险。埃文斯综合征中风的治疗由于其稀有性和缺乏明确的指南,仍然具有挑战性,需要个体化的治疗方法。未来的前瞻性研究有必要确定埃文斯综合征中风后需要抗凝剂二级预防的最佳患者人群。© EFIM 2024。
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.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.© EFIM 2024.