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补体介导的自身免疫性溶血性贫血作为军团病的初始表现。

Complement-mediated autoimmune haemolytic anaemia as an initial presentation of Legionnaires' disease.

机构信息

Internal Medicine, Rashid Hospital, Dubai, UAE

Internal Medicine, Rashid Hospital, Dubai, UAE.

出版信息

BMJ Case Rep. 2021 Aug 3;14(8):e243023. doi: 10.1136/bcr-2021-243023.

Abstract

A 42-year-old diabetic man presented to the hospital with severe sepsis and multiorgan dysfunction. A probable respiratory source of sepsis was suspected because of suggestive clinical and radiological findings. He was critically ill and was therefore admitted to intensive care for further management including ventilatory support and renal replacement therapy. He was also found to have marked anaemia requiring multiple blood transfusions with clinical and laboratory evidence pointing towards severe haemolysis. Further workup for the aetiology of pneumonia established a diagnosis of Legionella by confirmatory tests namely legionella antigen in the urine and exponentially rising serum antibody titres. The cause for the severe haemolysis was found to be complement-mediated autoimmune haemolysis as determined by direct antiglobulin test positive for complement components C3 and negative for IgG. Such clinically significant autoimmune haemolysis as a presenting feature, rather than a late complication, has never before been reported in the literature.

摘要

一位 42 岁的糖尿病男性因严重脓毒症和多器官功能障碍到医院就诊。由于有提示性的临床和影像学发现,怀疑脓毒症的可能来源是呼吸道。他病情危急,因此被收入重症监护病房进行进一步治疗,包括通气支持和肾脏替代治疗。他还被发现严重贫血,需要多次输血,临床和实验室证据表明存在严重溶血性贫血。进一步检查肺炎的病因,通过确认试验(即尿液中的军团菌抗原和血清抗体滴度呈指数上升)确定了军团菌的诊断。严重溶血性贫血的原因被确定为补体介导的自身免疫性溶血性贫血,直接抗球蛋白试验阳性,补体成分 C3 阳性,IgG 阴性。这种以自身免疫性溶血性贫血为主要表现,而非晚期并发症的临床表现,在文献中从未有过报道。

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