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      PLASMIC积分对血栓性血小板减少性紫癜和弥散性血管内凝血鉴别的临床价值 Translated title: The diagnostic value of PLASMIC score in the discrimination between thrombotic thrombocytopenic purpura and disseminated intravascular coagulation

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          Abstract

          目的

          比较PLASMIC积分在血栓性血小板减少性紫癜(TTP)和弥散性血管内凝血(DIC)患者中的差异,评价该积分系统对于两种疾病鉴别诊断的临床价值。

          方法

          回顾性分析24例TTP和41例DIC患者的临床资料,以及血小板计数、平均红细胞体积、间接胆红素、肌酐和凝血酶原时间-国际标准化比值,统计PLASMIC积分。

          结果

          根据PLASMIC积分预测伴有ADAMTS13严重缺乏的TTP的危险度分层,24例TTP患者中,3例(12.5%)存在中度风险,21例(87.5%)存在高度风险。而在41例DIC患者中,38例(92.7%)存在低度风险,2例(4.9%)存在中度风险,仅有1例(2.4%)患者存在高度风险。在65例患者中,PLASMIC积分达高度风险对于TTP诊断的敏感性为87.5%,特异性为97.6%。

          结论

          PLASMIC积分系统高度风险的患者诊断为TTP的可能性大。该积分系统对于TTP和DIC的鉴别诊断有较高的临床价值。

          Translated abstract

          Objective

          PLASMIC score was evaluated its value in differential diagnosis between the patients with thrombotic thrombocytopenic purpura (TTP) and those with disseminated intravascular coagulation (DIC).

          Methods

          Twenty-four patients with TTP and 41 cases with DIC were retrospectively analyzed in this study. The platelet count, average red blood cell volume, indirect bilirubin, creatinine and prothrombin time international normalised ratio were collected, and then PLASMIC scores were calculated.

          Results

          According to the risk classification of PLASMIC score, three (12.5%) TTP patients had moderate risk, and the rest 21 (87.5%) cases had high risk. In DIC patients, 92.7% cases were in low risk group, 4.9% at moderate risk, and only one case had high risk. Of these 65 patients, the sensitivity and the specificity to TTP of the high risk of the scoring system were 87.5% and 97.6%, respectively.

          Conclusion

          The patients with high risk of PLASMIC score correlated well with clinical TTP diagnosis. The scoring system showed to be an excellent diagnostic model to distinguish TTP patients from those with DIC.

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          Most cited references8

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          Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan.

          Thrombotic thrombocytopenic purpura (TTP) can rapidly progress into a life-threatening condition, thus the importance of appropriate diagnosis and treatment cannot be overstated. Until recently, TTP has mainly been diagnosed by clinical findings such as thrombocytopenia and non-immune hemolytic anemia. In addition to these clinical findings, however, reduced activity of a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) below 10% has been accepted internationally as a diagnostic criterion for TTP. In the present guidelines, we have taken all of these criteria into consideration. TTP is classified as acquired if the patient is positive for anti-ADAMTS13 autoantibodies, and as congenital if ADAMTS13 gene abnormalities are detected. Fresh-frozen plasma (FFP) transfusion is performed in patients with congenital TTP to supplement ADAMTS13. Plasma exchange therapy using FFP is conducted in patients with acquired TTP to supplement ADAMTS13 and remove anti-ADAMTS13 autoantibodies. To suppress autoantibody production, corticosteroid therapy may be administered in conjunction with plasma exchange. Recent reports show that the monoclonal anti-CD-20 antibody rituximab is effective in patients with refractory or relapsed TTP.
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            External validation of the PLASMIC score: a clinical prediction tool for thrombotic thrombocytopenic purpura diagnosis and treatment

            Essentials Severe ADAMTS-13 deficiency is key to thrombotic thrombocytopenic purpura (TTP) diagnosis. PLASMIC score predicts ADAMTS-13 deficiency in suspected TTP with high discrimination. PLASMIC score is more generalizable with fewer missing data than alternative clinical scores. PLASMIC score identifies a subgroup of patients lacking significant response to plasma exchange.
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              The PLASMIC score for thrombotic thrombocytopenic purpura

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                Author and article information

                Journal
                Zhonghua Xue Ye Xue Za Zhi
                Zhonghua Xue Ye Xue Za Zhi
                CJH
                Chinese Journal of Hematology
                Editorial office of Chinese Journal of Hematology (No. 288, Nanjing road, Heping district, Tianjin )
                0253-2727
                2707-9740
                October 2018
                : 39
                : 10
                : 812-816
                Affiliations
                [1]215006 苏州大学附属第一医院血液科,江苏省血液研究所,卫生部血栓与止血重点实验室,血液学协同创新中心(殷杰、余自强、孔丹青、王兆钺、苏健、张威、阮长耿),重症监护科(金钧、王俊),输血科(王雪明)
                Author notes
                通信作者:阮长耿,Email: cshcma@ 123456medmail.com.cn
                Corresponding author: Ruan Changgeng, Jiangsu Institute of Hematology, MOH Key Lab of Thrombosis and Hemostasis, The First Affiliated Hospital of Soochow University, Collaborative Innovation Center of Hematology, Soochow University, Suzhou 215006, China. Email: cshcma@ 123456medmail.com.cn
                Article
                cjh-39-10-812
                10.3760/cma.j.issn.0253-2727.2018.10.005
                7348283
                30369201
                533d1dae-730c-4240-aa1d-d570575c3068
                2018年版权归中华医学会所有Copyright © 2018 by Chinese Medical Association

                This work is licensed under a Creative Commons Attribution 3.0 License (CC-BY-NC). The Copyright own by Publisher. Without authorization, shall not reprint, except this publication article, shall not use this publication format design. Unless otherwise stated, all articles published in this journal do not represent the views of the Chinese Medical Association or the editorial board of this journal.

                History
                : 22 May 2018
                Funding
                基金项目:江苏省科教强卫工程-临床医学中心(YXZXA2016002);国家自然青年科学基金(81600105)
                Fund program: The Project of Invigorating Health Care through Science,Technology and Education, Jiangsu Provincial Key Medical Center(YXZXA2016002); National Natural Science Foundation of China(81600105)
                Categories
                论著

                plasmic积分,血栓性血小板减少性紫癜,弥散性血管性内凝血,诊断,plasmic score,thrombotic thrombocytopenic pupura,disseminated intravascular coagulation,diagnosis

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