Risk factors of death or chronic renal replacement therapy requirements in patients with thrombotic microangiopathies without ADAMTS-13 deficiency

IF 2.3 3区 医学 Q2 HEMATOLOGY
Miguel G. Uriol-Rivera, Bernardo López Andrade, Antonio Mas Bonet, Aina Obrador Mulet, Carmen Ballester Ruiz, Leonor Periañez Parraga, Javier Lumbreras, José Ignacio Ayestarán Rota, Mireia Ferreruela Servalos, Joana Ferrer Balaguer, Lucio Pallares Ferreres, María Jose Picado Valles, Rosa María Ruíz de Gopegui Valero, Susana Tarongi Sanchez, Ana Garcia Martin, Juan Rodríguez Garcia, Cristina Gomez Cobo, Daniel Ramis-Cabrer, the Son Espases Multidisciplinary Team for the management of Thrombotic Microangiopathy
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引用次数: 0

Abstract

Thrombotic microangiopathy (TMA), characterized by microangiopathic hemolytic anemia, thrombocytopenia, and multisystem organ dysfunction, is a life-threatening disease. Patients with TMA who do not exhibit a severe ADAMTS-13 deficiency (defined as a disintegrin-like and metalloprotease with thrombospondin type 1 motif no. 13 activity ≥10%: TMA-13n) continue to experience elevated mortality rates. This study explores the prognostic indicators for augmented mortality risk or necessitating chronic renal replacement therapy (composite outcome: CO) in TMA-13n patients. We included 42 TMA-13n patients from January 2008 to May 2018. Median age of 41 years and 60% were female. At presentation, 62% required dialysis, and 57% warranted intensive care unit admission. CO was observed in 45% of patients, including a 9-patient mortality subset. Multivariate logistic regression revealed three independent prognostic factors for CO: early administration of eculizumab (median time from hospitalization to eculizumab initiation: 5 days, range 0–19 days; odds ratio [OR], 0.14; 95% confidence interval [CI], 0.02–0.94), presence of neuroradiological lesions (OR, 6.67; 95% CI, 1.12–39.80), and a PLASMIC score ≤4 (OR, 7.39; 95% CI, 1.18–46.11). In conclusion, TMA-13n patients exhibit a heightened risk of CO in the presence of low PLASMIC scores and neuroradiological lesions, while early eculizumab therapy was the only protective factor.

无 ADAMTS-13 缺乏症的血栓性微血管病患者死亡或需要慢性肾脏替代治疗的风险因素。
血栓性微血管病(TMA)以微血管病性溶血性贫血、血小板减少和多系统器官功能障碍为特征,是一种危及生命的疾病。TMA 患者如果没有表现出严重的 ADAMTS-13 缺乏症(定义为具有凝血酶原 1 型 13 号基序的崩解素样和金属蛋白酶活性≥10%:TMA-13n)的患者死亡率持续升高。本研究探讨了 TMA-13n 患者死亡风险增加或需要进行慢性肾脏替代治疗(综合结果:CO)的预后指标。我们纳入了 2008 年 1 月至 2018 年 5 月的 42 名 TMA-13n 患者。中位年龄为 41 岁,60% 为女性。发病时,62%的患者需要透析,57%的患者需要入住重症监护室。45%的患者观察到 CO,其中有 9 名患者死亡。多变量逻辑回归显示,CO 有三个独立的预后因素:尽早使用依库珠单抗(从住院到开始使用依库珠单抗的中位时间为 5 天,范围为 0-19 天;从住院到开始使用依库珠单抗的中位时间为 5 天,范围为 0-19 天):5天,范围0-19天;比值比[OR],0.14;95%置信区间[CI],0.02-0.94)、神经放射学病变的存在(OR,6.67;95% CI,1.12-39.80)和PLASMIC评分≤4(OR,7.39;95% CI,1.18-46.11)。总之,TMA-13n患者在PLASMIC评分较低和神经放射学病变的情况下发生CO的风险较高,而早期依库珠单抗治疗是唯一的保护因素。
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来源期刊
CiteScore
5.50
自引率
0.00%
发文量
168
审稿时长
4-8 weeks
期刊介绍: European Journal of Haematology is an international journal for communication of basic and clinical research in haematology. The journal welcomes manuscripts on molecular, cellular and clinical research on diseases of the blood, vascular and lymphatic tissue, and on basic molecular and cellular research related to normal development and function of the blood, vascular and lymphatic tissue. The journal also welcomes reviews on clinical haematology and basic research, case reports, and clinical pictures.
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