Douglas Kenneth W, Pollock Kevin G J, Young David, Catlow Jamie, Green Rachel
Clinical Apheresis Unit, Beatson West of Scotland Cancer Centre, Glasgow, United Kingdom.
J Clin Apher. 2010;25(2):47-53. doi: 10.1002/jca.20226.
Thrombotic microangiopathies are rare conditions characterized by microangiopathic hemolytic anemia, microthrombi, and multiorgan insult. The disorders, which include hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, are often acute and life threatening. We report a retrospective analysis of 65 patients presenting to our institution from 1997 to 2008 with all forms of thrombotic microangiopathy. Therapeutic plasma exchange was a requirement for analysis and 65 patients were referred to our institution; 66% of patients were female and median age at presentation was 52 years. Bacterial infection was the most commonly identified etiologic factor and in the multivariate model was the only significant variable associated with survival outcome (odds ratio 5.1, 95% confidence interval, 1.2-21.7). As infection can be considered a common trigger event for thrombotic microangiopathy, patients with hepatobiliary sepsis may benefit from elective cholecystectomy. We conclude that bacterial infection frequently triggers TTP and other thrombotic microangiopathies in patients with preexisting risk factors and propose a model for the development of these syndromes.
血栓性微血管病是一类罕见疾病,其特征为微血管病性溶血性贫血、微血栓形成及多器官损害。这些疾病包括溶血性尿毒症综合征和血栓性血小板减少性紫癜,通常呈急性发作且危及生命。我们报告了1997年至2008年期间在我院就诊的65例各种形式血栓性微血管病患者的回顾性分析。治疗性血浆置换是分析的必要条件,65例患者被转诊至我院;66%的患者为女性,就诊时的中位年龄为52岁。细菌感染是最常见的病因,在多变量模型中是与生存结局相关的唯一显著变量(比值比5.1,95%置信区间,1.2 - 21.7)。由于感染可被视为血栓性微血管病的常见触发事件,患有肝胆系统败血症的患者可能从择期胆囊切除术中获益。我们得出结论,细菌感染经常在已有危险因素的患者中触发血栓性血小板减少性紫癜和其他血栓性微血管病,并提出了这些综合征的发病模型。