Service de Médecine Intensive-Réanimation, Hôpital Saint-Louis, APHP, Paris, France.
Université de Paris, Paris, France.
Crit Care Med. 2021 Oct 1;49(10):e931-e940. doi: 10.1097/CCM.0000000000005164.
To describe short- and long-term neurologic prognosis of patients with thrombotic thrombocytopenic purpura and to identify clusters associated with evolution.
Prospective French cohort.
ICU in a reference center.
All consecutive patients with newly diagnosed thrombocytopenic purpura.
Comprehensive clinical, biological, and radiological evaluation at admission. Neurocognitive recovery was assessed using Glasgow Outcome Scale (range 1-5, with 1 representing death and 5 representing no or minimal neurologic deficit).
Among the 130 newly diagnosed patients with thrombocytopenic purpura, 108 (83%; age 43 [30-52]; 73% women) presented with neurologic signs, including headaches (51%), limb weakness, paresthesia, and/or aphasia (49%), pyramidal syndrome (30%), decreased consciousness (20%), seizure (19%), cognitive impairment (34%), cerebellar syndrome (18%), and visual symptoms (20%). A hierarchical cluster analysis identified three distinct groups of patients. Cluster 1 included younger patients (37 [27-48], 41 [32-52], and 48 [35-54], in clusters 1, 2 and 3, respectively; p = 0.045), with a predominance of headaches (75%, 27%, and 36%; p < 0.0001). Cluster 2 patients had ataxic gait and cerebellar syndrome (77%, 0%, and 0%; p < 0.0001) and dizziness (50%, 0%, and 0%; p < 0.0001). Cluster 3 included patients with delirium (36%, 0%, and 9%; p < 0.0001), obtundation (58%, 0%, and 24%; p < 0.0001), and seizure (36%, 0%, and 14%; p < 0.0001). Acute kidney injury was 32%, 68%, and 77%, in clusters 1, 2, and 3, respectively (p < 0.0001). The three clusters did not differ for other biological or brain imaging. After a median follow-up of 34 months (12-71 mo), 100 patients (93%) were alive with full neurocognitive recovery (i.e., Glasgow Outcome Scale score 5) in 89 patients (89%). Patients from cluster 1 more frequently exhibited full recovery (Glasgow Outcome Scale score of 5) compared with clusters 2 and 3, (44 [98%], 13 [65%], and 21 [60%] at 3 mo; p < 0.0001), (44 [100%], 15 [68%], and 23 [69%] at 6 mo; p < 0.0001), and (40 [100%], 15 [79%], and 20 [57%] at 1 yr; p < 0.0001).
Initial clinical neurologic evaluation in thrombocytopenic purpura patients distinguishes three groups of patients with different clinical and functional outcomes.
描述血栓性血小板减少性紫癜患者的短期和长期神经预后，并确定与疾病进展相关的聚类。
前瞻性法国队列研究。
参考中心的 ICU。
所有新诊断为血小板减少性紫癜的连续患者。
入院时进行全面的临床、生物学和影像学评估。使用格拉斯哥预后量表（范围 1-5，1 代表死亡，5 代表无或最小神经缺陷）评估神经认知恢复情况。
在 130 名新诊断的血小板减少性紫癜患者中，108 名（83%；年龄 43[30-52]；73%为女性）出现了神经症状，包括头痛（51%）、肢体无力、感觉异常和/或失语（49%）、锥体束征（30%）、意识下降（20%）、癫痫发作（19%）、认知障碍（34%）、小脑综合征（18%）和视觉症状（20%）。层次聚类分析确定了三个不同的患者组。第 1 组包括年轻患者（37[27-48]、41[32-52]和 48[35-54]，分别在第 1、2 和 3 组中；p=0.045），以头痛为主（75%、27%和 36%；p<0.0001）。第 2 组患者有共济失调步态和小脑综合征（77%、0%和 0%；p<0.0001）和头晕（50%、0%和 0%；p<0.0001）。第 3 组包括出现谵妄（36%、0%和 9%；p<0.0001）、意识模糊（58%、0%和 24%；p<0.0001）和癫痫发作（36%、0%和 14%；p<0.0001）的患者。第 1、2 和 3 组的急性肾损伤分别为 32%、68%和 77%（p<0.0001）。这三个聚类在其他生物学或脑部成像方面没有差异。中位随访 34 个月（12-71 个月）后，100 名患者（93%）存活，89 名患者（89%）完全恢复神经认知功能（即格拉斯哥预后量表评分为 5）。与聚类 2 和 3 相比，聚类 1 的患者更频繁地表现出完全恢复（格拉斯哥预后量表评分为 5），（3 个月时 44[98%]、13[65%]和 21[60%]；p<0.0001），（6 个月时 44[100%]、15[68%]和 23[69%]；p<0.0001）和（1 年时 40[100%]、15[79%]和 20[57%]；p<0.0001）。
血小板减少性紫癜患者的初始临床神经评估可区分具有不同临床和功能结局的三组患者。
