Division of Hematology, Department of Medicine, The Ohio State University College of Medicine, Columbus, OH.
Department of Medicine.
Blood Adv. 2022 Feb 22;6(4):1264-1270. doi: 10.1182/bloodadvances.2020004169.
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) survivors experience high rates of adverse health sequelae and increased mortality over long-term follow-up. We conducted this multicenter cohort study to evaluate long-term mortality and causes of death in iTTP survivors. Between 2003 and 2020, 222 patients were enrolled in the Ohio State University and Johns Hopkins TTP registries and followed for a median of 4.5 (interquartile range [IQR], 75 0.4-11.5) years. Nine patients died during their first iTTP episode, and 29 patients died during follow-up. Mortality rate was 1.8 times higher than expected from an age-, sex-, and race-adjusted reference population. Cardiovascular disease was a leading primary cause of death (27.6%) tied with relapsed iTTP (27.6%), followed by malignancy (20.7%), infection (13.8%), and other causes (10.3%). Male sex (hazard ratio [HR], 3.74; 95% confidence interval [CI], 1.65-8.48), increasing age (HR, 1.04; 95% CI, 1.01-1.07), and number of iTTP episodes (HR, 1.10; 95% CI, 1.01-1.20) were associated with mortality in a model adjusted for African American race (HR, 0.70; 95% CI, 0.30-1.65), hypertension (HR, 0.47; 95% CI, 0.20-1.08), chronic kidney disease (HR 1.46; 95% CI, 0.65-3.30), and site (HR, 1.46; 95% CI, 0.64-3.30). There was a trend toward shorter survival in patients with lower ADAMTS13 activity during remission (P = .078). Our study highlights the need for survivorship care and investigation focused on cardiovascular disease and early mortality in TTP survivors.
免疫介导性血栓性血小板减少性紫癜（iTTP）幸存者在长期随访中经历高比率的不良健康后果和死亡率增加。我们进行了这项多中心队列研究，以评估 iTTP 幸存者的长期死亡率和死亡原因。在 2003 年至 2020 年间，共有 222 名患者入组俄亥俄州立大学和约翰霍普金斯 TTP 登记处，并随访中位数为 4.5 年（四分位距[IQR]，75 0.4-11.5）。9 名患者在首次 iTTP 发作期间死亡，29 名患者在随访期间死亡。死亡率比根据年龄、性别和种族调整的参考人群高 1.8 倍。心血管疾病是主要的原发性死亡原因（27.6%），与复发 iTTP（27.6%）并列，其次是恶性肿瘤（20.7%）、感染（13.8%）和其他原因（10.3%）。男性（危险比[HR]，3.74；95%置信区间[CI]，1.65-8.48）、年龄增加（HR，1.04；95%CI，1.01-1.07）和 iTTP 发作次数（HR，1.10；95%CI，1.01-1.20）在调整非裔美国人种族（HR，0.70；95%CI，0.30-1.65）、高血压（HR，0.47；95%CI，0.20-1.08）、慢性肾脏病（HR 1.46；95%CI，0.65-3.30）和发病部位（HR 1.46；95%CI，0.64-3.30）的模型中与死亡率相关。在缓解期 ADAMTS13 活性较低的患者中，生存趋势较短（P=.078）。我们的研究强调了需要进行生存者护理，并需要关注 TTP 幸存者的心血管疾病和早期死亡率。
