依库珠单抗治疗阵发性夜间血红蛋白尿和重症肌无力罕见并发症中发生的大量溶血

IF 16.4 1区 化学 Q1 CHEMISTRY, MULTIDISCIPLINARY Accounts of Chemical Research Pub Date : 2024-04-19 DOI:10.3390/hematolrep16020025
Ráhel Réka Bicskó, Árpád Illés, Zsuzsanna Hevessy, G. Ivády, György Kerekes, Gábor Méhes, T. Csépány, L. Gergely
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引用次数: 0

摘要

重症肌无力(MG)和阵发性夜间血红蛋白尿(PNH)同时出现的情况非常罕见,迄今为止只发表过一例。我们报告了一名 63 岁的白种女性患者,她在 43 岁时被诊断出患有重症肌无力。同时还发现了胸腺瘤,因此进行了手术切除,结果近 20 年来病情得到了合理控制。患者开始出现轻微溶血,随后肌无力症状进展,因此开始接受免疫抑制治疗。由于病情进展和呼吸衰竭,患者接受了浆液吸出术,并停止了呼吸机支持。患者出现了明显的溶血,诊断性检查证实其患有 III 型 PNH 细胞。她的肌无力症状加重,不得不再次开始机械通气,由于呼吸性酸中毒,发生了大量溶血。经过两次血浆置换治疗后,患者接受了 600 毫克的依库珠单抗治疗,溶血很快得到控制。第二剂治疗后,患者被拔掉了气管。但由于无法咳嗽,她再次出现呼吸衰竭和肺炎-败血症,导致患者死亡。本病例强调了这两种严重疾病之间罕见的关联,以及涉及补体系统的类似免疫介导病理生理学机制。
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Eculizumab Treatment of Massive Hemolysis Occurring in a Rare Co-Existence of Paroxysmal Nocturnal Hemoglobinuria and Myasthenia Gravis
The co-occurrence of myasthenia gravis (MG) and paroxysmal nocturnal hemoglobinuria (PNH) is rare; only one case has been published so far. We report a 63-year-old Caucasian female patient who was diagnosed with MG at the age of 43. Thymoma was also detected, and so it was surgically resected, which resulted in reasonable disease control for nearly 20 years. Slight hemolysis began to emerge, and then myasthenia symptoms progressed, so immunosuppressive therapy was started. Due to progressive disease and respiratory failure, the patient underwent plasmapheresis, and ventilatory support was stopped. Marked hemolysis was present, and diagnostic tests confirmed PNH with type III PNH cells. Her myasthenia symptoms aggravated, mechanical ventilation had to be started again, and due to the respiratory acidosis, massive hemolysis occurred. After two plasmapheresis sessions, the patient received eculizumab at 600 mg, resulting in prompt hemolysis control. After the second dose of the treatment, the patient was extubated. Still, due to their inability to cough, she developed another respiratory failure and pneumonia–sepsis, resulting in the patient’s death. This case highlights the rare association between these two serious diseases and similar immune-mediated pathophysiology mechanisms involving the complement system.
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来源期刊
Accounts of Chemical Research
Accounts of Chemical Research 化学-化学综合
CiteScore
31.40
自引率
1.10%
发文量
312
审稿时长
2 months
期刊介绍: Accounts of Chemical Research presents short, concise and critical articles offering easy-to-read overviews of basic research and applications in all areas of chemistry and biochemistry. These short reviews focus on research from the author’s own laboratory and are designed to teach the reader about a research project. In addition, Accounts of Chemical Research publishes commentaries that give an informed opinion on a current research problem. Special Issues online are devoted to a single topic of unusual activity and significance. Accounts of Chemical Research replaces the traditional article abstract with an article "Conspectus." These entries synopsize the research affording the reader a closer look at the content and significance of an article. Through this provision of a more detailed description of the article contents, the Conspectus enhances the article's discoverability by search engines and the exposure for the research.
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