Background: Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder resulting in organ damage including ischemic strokes. We sought to characterize the neuroimaging patterns of stroke in a large cohort of patients with immune-mediated TTP (iTTP) and determined their associations with clinical and laboratory parameters and outcomes. Methods: We analyzed the Alabama TTP Registry who had laboratory confirmation of acute iTTP. We reviewed the neuroimaging patterns of those with ischemic stroke on MRI, clinical information, and laboratory results. Small ischemic strokes were ≤20 mm in their maximum diameter in the axial plane. Large ischemic strokes were #x3e;20 mm. Student t test, Mann-Whitney U test, and χ² test were all used for data analysis. Results: Of 108 iTTP patients, 21 had ischemic stroke on neuroimaging. The median platelet count in these patients was 12 × 10⁹/L (interquartile range, IQR, 8.8–21 × 10⁹/L), plasma ADAMTS13 activity 1.8 U/dL (IQR 0–4.5 U/dL), and the mean plasma level of anti-ADAMTS13 IgG was 6,595.8 U/mL (SD 3,448.9 U/mL). Comparison between patients with large ischemic strokes (n = 10) and small ischemic strokes (n = 11) revealed that patients with small stroke were older (p = 0.043) and had higher plasma levels of citrullinated histone 3 (p = 0.006) and histone/DNA complex (p = 0.014) than those with large strokes. There were no significant differences between 2 stroke groups in mortality or exacerbation. Conclusions: iTTP patients can present with large ischemic strokes and are usually younger. Further research should be performed in assessing different etiologies of iTTP-associated stroke based on neutrophil extracellular trap formation biomarkers (e.g., histone markers) seen in small ischemic stroke.
Cerebrovasc Dis Extra 2021;11:29–36

中文翻译：

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伴有大，小缺血性卒中的血栓性血小板减少性紫癜患者的生物标志物鉴定

背景：血栓性血小板减少性紫癜（TTP）是一种罕见的血液病，会导致器官损害，包括缺血性中风。我们试图表征一大批免疫介导的TTP（iTTP）患者的卒中的神经影像学特征，并确定他们与临床和实验室参数及结果的关联。方法：我们分析了阿拉巴马州TTP注册中心，该注册中心已对急性iTTP进行了实验室确认。我们在MRI，临床信息和实验室结果中回顾了缺血性卒中患者的神经影像学模式。小缺血性卒中的轴向最大直径≤20 mm。较大的缺血性卒中为＃x3e; 20 mm。学生吨检验，Mann-Whitney U检验，和χ ²测试全部用于数据分析。结果：在108名iTTP患者中，有21名在神经影像学上发生了缺血性中风。这些患者的血小板中位数为12×10 ⁹ / L（四分位间距，IQR，8.8–21×10 ⁹ / L），血浆ADAMTS13活性为1.8 U / dL（IQR 0–4.5 U / dL），且均值抗ADAMTS13 IgG的血浆水平为6,595.8 U / mL（SD 3,448.9 U / mL）。比较大缺血性卒中（n = 10）和小缺血性卒中（n = 11）的患者，发现小卒中的患者年龄较大（p = 0.043），瓜氨酸化组蛋白3（p = 0.006）和组蛋白的血浆水平更高/ DNA复合物（p= 0.014）。两个卒中组的死亡率或病情加重之间无显着差异。结论： iTTP患者可出现较大的缺血性卒中，且通常较年轻。应基于在小缺血性卒中中发现的中性粒细胞胞外陷阱形成生物标志物（例如组蛋白标志物），评估iTTP相关中风的不同病因，应进行进一步的研究。
Cerebrovasc Dis Extra 2021; 11：29–36