Department of Transfusion Medicine, West China Hospital of Sichuan University, Chengdu, China.
Department of Transfusion Medicine, West China Hospital of Sichuan University, Chengdu, China.
Transfus Apher Sci. 2023 Jun;62(3):103661. doi: 10.1016/j.transci.2023.103661. Epub 2023 Mar 1.
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA), and therapeutic plasma exchange (TPE) is currently the standard treatment. However, TPE sometimes cannot be implemented. The aim of this study was to systematically review patients with a first TTP episode who were treated without TPE.
The PubMed, Embase, Web of Science and Cochrane Library databases were searched by two investigators independently to collect case reports and clinical studies on TTP patients treated without TPE. After removing duplicate records and records that did not meet the inclusion criteria, the patients' data of eligible studies, including the basic characteristics, treatment regimens, and outcomes were extracted for further analysis.
A total of 5338 potentially relevant original studies were identified, from which 21 studies, including 14 cases, 3 case series and 4 retrospective studies, met eligibility requirements and were included. Treatment regimens in the absence of TPE were found to vary based on individual information. Most patients recovered, with normal platelet counts and ADAMT13 activity at discharge. In the meta-analysis of retrospective studies, the TPE-free group had no higher mortality than the TPE-treated group.
Our study shows that TPE-free treatment may not increase the mortality of TTP patients, which provides a new treatment concept for patients with first episodes of TTP. However, the current evidence is not high due to the lack of randomized controlled trials, so more well-designed prospective clinical trials are warranted to investigate the safety and efficacy of TPE-free treatment regimens in TTP patients.
血栓性血小板减少性紫癜（TTP）是一种血栓性微血管病，目前标准治疗方法是治疗性血浆置换（TPE）。然而，有时无法进行 TPE。本研究旨在系统综述未接受 TPE 治疗的初发 TTP 患者。
由两名研究者独立检索 PubMed、Embase、Web of Science 和 Cochrane Library 数据库，收集未接受 TPE 治疗的 TTP 患者的病例报告和临床研究。去除重复记录和不符合纳入标准的记录后，提取合格研究的患者数据，包括基本特征、治疗方案和结局，以便进一步分析。
共确定了 5338 篇潜在相关的原始研究，其中 21 项研究符合纳入标准，包括 14 例病例、3 个病例系列和 4 项回顾性研究，共纳入 14 例患者。在缺乏 TPE 的情况下，治疗方案根据个体信息而有所不同。大多数患者康复，出院时血小板计数和 ADAMT13 活性正常。在回顾性研究的荟萃分析中，TPE 免费组的死亡率不比 TPE 治疗组高。
我们的研究表明，TPE 免费治疗不会增加 TTP 患者的死亡率，为初发 TTP 患者提供了一种新的治疗理念。然而，由于缺乏随机对照试验，目前的证据水平不高，因此需要更多设计良好的前瞻性临床试验来研究 TPE 免费治疗方案在 TTP 患者中的安全性和疗效。
