Lee Chun-Hui, Huang Yi-Ching, Li Sin-Syue, Hsu Ya-Ting, Chen Ya-Ping, Chen Tsai-Yun
Institute of Clinical Medicine, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
Department of Oncology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
Front Med (Lausanne). 2022 May 9;9:893273. doi: 10.3389/fmed.2022.893273. eCollection 2022.
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder caused by severe ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13) deficiency (activity <10%). Urgent intervention based on the timely evaluation of ADAMTS13 level is crucial to guide optimal therapy. The recently developed PLASMIC score based on seven items allows the rapid identification of patients at high risk for TTP due to severe ADAMTS13 deficiency. This retrospective study included 31 hospitalized patients with suspicious thrombotic microangiopathy in National Cheng Kung University Hospital from December 2016 to July 2021. Data on ADAMTS13 activity and medical and laboratory information were retrieved from medical records. The PLASMIC score could be calculated in 24 of the 31 patients with available data, and the final cohort was stratified according to the 7-point PLASMIC score. All patients with high PLASMIC score (6-7) exhibited severe ADAMTS13 deficiency (activity ≤10%). One patient with a brain tumor and a PLASMIC score of 6 did not have severe ADAMTS13 activity of ≤10%. The patients in the intermediate- and low risk groups (PLASMIC scores of 5 and 0-4, respectively) exhibited ADAMTS13 activities of above 10%. Given the role of prompt diagnosis in the timely delivery of appropriate therapy, these findings confirm and strengthen the predictive value of the PLASMIC score in patients at high risk for TTP due to severe ADAMTS13 deficiency.
血栓性血小板减少性紫癜(TTP)是一种由严重的ADAMTS13(含Ⅰ型血小板反应蛋白基序的解聚素和金属蛋白酶13)缺乏(活性<10%)引起的危及生命的疾病。基于ADAMTS13水平的及时评估进行紧急干预对于指导最佳治疗至关重要。最近开发的基于七个项目的PLASMIC评分能够快速识别因严重ADAMTS13缺乏而患TTP高风险的患者。这项回顾性研究纳入了2016年12月至2021年7月在国立成功大学医院住院的31例疑似血栓性微血管病患者。从病历中获取ADAMTS13活性以及医疗和实验室信息的数据。在31例有可用数据的患者中,24例可计算PLASMIC评分,并根据7分的PLASMIC评分对最终队列进行分层。所有PLASMIC评分高(6 - 7分)的患者均表现出严重的ADAMTS13缺乏(活性≤10%)。1例患有脑肿瘤且PLASMIC评分为6分的患者,其ADAMTS13活性并未严重至≤10%。中低风险组(PLASMIC评分分别为5分和0 - 4分)的患者ADAMTS13活性高于10%。鉴于及时诊断在适时提供适当治疗中的作用,这些发现证实并强化了PLASMIC评分在因严重ADAMTS13缺乏而患TTP高风险患者中的预测价值。