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伴有干燥综合征的血栓性血小板减少性紫癜患者出现非典型后部可逆性脑病综合征

Atypical posterior reversible encephalopathy syndrome in a case of thrombotic thrombocytopenic purpura with Sjogren's syndrome.

作者信息

Hegde Rahul, Ismail Rasha, Vegunta Aishwariya, Megahed Ayah, Sharma Pranav, Rosovsky Mark

机构信息

Department of Radiology, Yale New Haven Health System, Bridgeport Hospital, Bridgeport, CT.

Department of Radiology, University of Minnesota Medical Center, Minneapolis, MIN.

出版信息

Radiol Case Rep. 2021 Apr 19;16(6):1552-1556. doi: 10.1016/j.radcr.2021.03.035. eCollection 2021 Jun.

DOI:10.1016/j.radcr.2021.03.035
PMID:33981376
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8082195/
Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare hematologic disorder characterized by thrombotic microangiopathy. Neurologic symptoms are frequently seen in its presentation and the most common finding on neuroimaging of TTP is posterior reversible encephalopathy syndrome (PRES). Acute strokes, hemorrhages and atypical PRES are uncommonly seen. Our case reports the clinical and imaging details of a young male patient with TTP and Sjogren's syndrome, who made a complete recovery after aggressive plasmapheresis and immunosuppressive therapy with resolution of the imaging findings of PRES on follow up brain MR imaging. We briefly review the literature for the spectrum of imaging findings that can be seen on brain MRI with TTP.

摘要

血栓性血小板减少性紫癜(TTP)是一种以血栓性微血管病为特征的罕见血液系统疾病。神经系统症状在其临床表现中较为常见,TTP神经影像学最常见的表现是后部可逆性脑病综合征(PRES)。急性中风、出血和非典型PRES则较为少见。我们的病例报告了一名患有TTP和干燥综合征的年轻男性患者的临床和影像学细节,该患者在积极进行血浆置换和免疫抑制治疗后完全康复,随访脑部磁共振成像显示PRES的影像学表现消失。我们简要回顾了有关TTP患者脑部MRI可见的影像学表现谱的文献。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6590/8082195/8108bc1c434b/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6590/8082195/1c47c750504d/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6590/8082195/beb5c7838696/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6590/8082195/8108bc1c434b/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6590/8082195/1c47c750504d/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6590/8082195/beb5c7838696/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6590/8082195/8108bc1c434b/gr3.jpg

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Validation of the PLASMIC score for predicting ADAMTS13 activity <10% in patients with suspected thrombotic thrombocytopenic purpura in Alberta, Canada.验证 PLASMIC 评分在预测加拿大阿尔伯塔省疑似血栓性血小板减少性紫癜患者 ADAMTS13 活性 <10%方面的准确性。
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