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本文引用的文献

1
Case Report of Thrombotic Thrombocytopenic Purpura in a Previously Healthy Adult.一名既往健康成年人的血栓性血小板减少性紫癜病例报告。
J Educ Teach Emerg Med. 2021 Jan 15;6(1):V1-V4. doi: 10.21980/J8VK9M. eCollection 2021 Jan.
2
Role of the PLASMIC Score in the Management of Thrombotic Thrombocytopenic Purpura.血浆评分在血栓性血小板减少性紫癜治疗中的作用
Cureus. 2023 Mar 15;15(3):e36188. doi: 10.7759/cureus.36188. eCollection 2023 Mar.
3
Plasma exchange and thrombotic microangiopathies: From pathophysiology to clinical practice.血浆置换与血栓性微血管病:从病理生理学到临床实践。
Transfus Apher Sci. 2020 Dec;59(6):102990. doi: 10.1016/j.transci.2020.102990. Epub 2020 Nov 19.
4
Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura.卡普拉珠单抗治疗获得性血栓性血小板减少性紫癜。
N Engl J Med. 2019 Jan 24;380(4):335-346. doi: 10.1056/NEJMoa1806311. Epub 2019 Jan 9.

无神经系统受累的血栓性血小板减少性紫癜:一例报告及诊断与治疗策略综述

Thrombotic Thrombocytopenic Purpura Without Neurological Involvement: A Case Report and Review of the Diagnostic and Treatment Strategies.

作者信息

Daive Kapeel, Sreeneyasan Jegadis

机构信息

Medicine, Lancashire Teaching Hospitals NHS Foundation Trust, Preston, GBR.

Medicine, Royal Bolton Hospital, Bolton, GBR.

出版信息

Cureus. 2025 May 7;17(5):e83654. doi: 10.7759/cureus.83654. eCollection 2025 May.

DOI:10.7759/cureus.83654
PMID:40486424
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12143186/
Abstract

This case report describes the presentation of thrombotic thrombocytopenic purpura (TTP) without neurological involvement in a 48-year-old woman. TTP is a relatively rare, life-threatening condition that comprises thrombotic microangiopathy (TMA) and enzymatic dysfunction of ADAMTS13 (a disintegrin-like metalloproteinase with thrombospondin motif type 1, member 13, which regulates platelet aggregation). Another similar condition that presents with TMA in adults is atypical hemolytic uremic syndrome. It is challenging to differentiate between the two conditions clinically, especially in the absence of neurological symptoms. However, plasma exchange is the initial lifesaving treatment for both in acute situations. Our patient had a history of bruising, which was evident in clinical examination, without any other positive findings. However, ADAMTS13 activity was 0%, and the platelet count was 4 × 10⁹/L on arrival. Timely investigation and treatment not only saved her life but also reduced end-organ damage. This report provides an overview of the acute presentation of TTP, positive clinical findings, investigations, diagnosis, treatment, and post-treatment outcomes.

摘要

本病例报告描述了一名48岁女性患血栓性血小板减少性紫癜(TTP)且无神经系统受累的情况。TTP是一种相对罕见的、危及生命的疾病,包括血栓性微血管病(TMA)和ADAMTS13(一种具有血小板反应蛋白基序的解整合素样金属蛋白酶13,可调节血小板聚集)的酶功能障碍。另一种在成人中表现为TMA的类似疾病是非典型溶血尿毒症综合征。临床上区分这两种疾病具有挑战性,尤其是在没有神经系统症状的情况下。然而,血浆置换是两种疾病在急性情况下的初始挽救生命的治疗方法。我们的患者有瘀伤病史,临床检查可见明显瘀伤,无任何其他阳性发现。然而,入院时ADAMTS13活性为0%,血小板计数为4×10⁹/L。及时的检查和治疗不仅挽救了她的生命,还减少了终末器官损伤。本报告概述了TTP的急性表现、阳性临床发现、检查、诊断、治疗及治疗后结果。