Department of Neurology, The University of Alabama at Birmingham, Birmingham, Alabama, USA,
Department of Pathology, The University of Alabama at Birmingham, Birmingham, Alabama, USA.
Cerebrovasc Dis Extra. 2021;11(1):29-36. doi: 10.1159/000513574. Epub 2021 Feb 18.
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.
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 >20 mm. Student t test, Mann-Whitney U test, and χ2 test were all used for data analysis.
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.
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.
血栓性血小板减少性紫癜（TTP）是一种罕见的血液疾病，可导致包括缺血性中风在内的器官损伤。我们旨在对大量免疫介导性 TTP（iTTP）患者的中风神经影像学模式进行特征描述，并确定其与临床和实验室参数及结果的相关性。
我们分析了阿拉巴马 TTP 登记处的实验室确诊的急性 iTTP 患者。我们对 MRI 显示缺血性中风的患者的神经影像学模式、临床信息和实验室结果进行了回顾。小面积缺血性中风的最大直径在轴向平面上小于 20mm；大面积缺血性中风的最大直径大于 20mm。所有数据分析均采用学生 t 检验、Mann-Whitney U 检验和 χ2 检验。
在 108 例 iTTP 患者中，21 例在神经影像学上出现缺血性中风。这些患者的血小板计数中位数为 12×109/L（四分位距，IQR，8.8-21×109/L），血浆 ADAMTS13 活性为 1.8 U/dL（IQR 0-4.5 U/dL），抗 ADAMTS13 IgG 的平均血浆水平为 6595.8 U/mL（标准差 3448.9 U/mL）。大缺血性中风组（n=10）和小缺血性中风组（n=11）患者之间的比较显示，小中风患者年龄更大（p=0.043），且血浆瓜氨酸化组蛋白 3（p=0.006）和组蛋白/DNA 复合物（p=0.014）水平更高。2 个中风组在死亡率或恶化方面无显著差异。
iTTP 患者可出现大的缺血性中风，且通常更年轻。应进一步研究基于中性粒细胞胞外诱捕网形成生物标志物（如组蛋白标志物）评估 iTTP 相关中风的不同病因。
