Goel Ruchika, King Karen E, Takemoto Clifford M, Ness Paul M, Tobian Aaron A R
Division of Pediatric Hematology.
Division of Transfusion Medicine, Department of Pathology, Johns Hopkins University, Baltimore, Maryland.
Transfusion. 2016 Jun;56(6):1451-8. doi: 10.1111/trf.13586. Epub 2016 Apr 15.
Despite proven efficacy and increased availability of therapeutic plasma exchange (TPE), mortality for patients with thrombotic thrombocytopenic purpura (TTP) remains high with a limited understanding of those at highest risk of death.
This study utilized the Nationwide Inpatient Sample (2007-2012) to derive a prognostic score for mortality in hospitalized TTP patients. Odds ratios of death with various putative risk factors adjusted for age, sex, and race were calculated (adjOR). Weighted mean of adjOR estimates were incorporated in a risk-stratified score.
Among 8203 hospitalizations with TTP as primary admission diagnosis who underwent TPE, 613 deaths were identified (all-cause mortality, 7.5%; median time-to-death, 9 days; interquartile range, 4-14 days). In multivariable logistic regression, arterial thrombosis (adjOR 6.7, 95% confidence interval [CI], 1.1-40.9), intracranial hemorrhage (adjOR, 6.1; 95% CI, 1.6-23.2), age at least 60 years (adjOR, 3.5; 95% CI, 2.1-5.6), renal failure (adjOR, 2.6; 95% CI, 1.5-4.5), ischemic stroke (adjOR, 2.4; 95% CI, 1.2-5.0), platelet (PLT) transfusions (adjOR, 2.2; 95% CI, 1.2-4.1), and myocardial infarction (adjOR, 2.3; 95% CI, 1.2-4.6) were significant independent predictors of mortality in TTP patients who underwent TPE. A prognostic weighted mortality prediction scoring system incorporating arterial thrombosis, intracranial hemorrhage, age, renal failure, ischemic stroke, PLT transfusion, and myocardial infarction showed very good discrimination and was predictive of 78.6% deaths.
Early and targeted therapy for high-risk individuals should be used to guide management of TTP patients for improved survival outcomes.
尽管治疗性血浆置换(TPE)已证实有效且可用性增加,但血栓性血小板减少性紫癜(TTP)患者的死亡率仍然很高,对死亡风险最高的患者了解有限。
本研究利用全国住院患者样本(2007 - 2012年)得出住院TTP患者的死亡预后评分。计算了针对年龄、性别和种族调整后的各种假定风险因素的死亡比值比(adjOR)。将adjOR估计值的加权平均值纳入风险分层评分。
在8203例以TTP作为主要入院诊断并接受TPE的住院病例中,确定有613例死亡(全因死亡率为7.5%;中位死亡时间为9天;四分位间距为4 - 14天)。在多变量逻辑回归中,动脉血栓形成(adjOR 6.7,95%置信区间[CI],1.1 - 40.9)、颅内出血(adjOR,6.1;95% CI,1.6 - 23.2)、年龄至少60岁(adjOR,3.5;95% CI,2.1 - 5.6)、肾衰竭(adjOR,2.6;95% CI,1.5 - 4.5)、缺血性中风(adjOR,2.4;95% CI,1.2 - 5.0)、血小板(PLT)输注(adjOR,2.2;95% CI,1.2 - 4.1)和心肌梗死(adjOR,2.3;95% CI,1.2 - 4.6)是接受TPE的TTP患者死亡的显著独立预测因素。一个纳入动脉血栓形成、颅内出血、年龄、肾衰竭、缺血性中风、PLT输注和心肌梗死的预后加权死亡率预测评分系统显示出很好的区分度,可预测78.6%的死亡病例。
应采用针对高危个体的早期和靶向治疗来指导TTP患者的管理,以改善生存结局。