42例血栓性血小板减少性紫癜患者的临床特点及预后分析

葛仁英.42例血栓性血小板减少性紫癜患者的临床特点及预后分析[J].内科急危重症杂志,2024,30(3):251-254

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DOI：10.11768/nkjwzzzz20240312

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基金项目:咸宁市中心医院湖北科技学院附属第一医院院级课题（2022XYB016）

作者	单位
葛仁英	咸宁市中心医院湖北科技学院附属第一医院

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中文摘要:

摘要目的：分析42例血栓性血小板减少性紫癜(TTP)患者的临床特征、实验室指标、治疗及转归，为临床提高诊治提供参考。方法：回顾性分析湖北省多家三甲医院诊断为TTP患者的临床资料，根据疾病转归分为存活组（23例）及死亡组（19例），比较2组患者的临床特点及不同血浆治疗方法疗效的差异，并通过计算普拉斯米克（PLASMIC）分数预测血管性血友病因子裂解酶（ADAMTS13）活性减少的精确度。结果：42例患者中，男16例(38.1%)，女26例(61.9%)；中位发病年龄62岁，平均(56.1±16.3)岁。 23例（54.8%）表现为“三联征”，18例（42.9%）表现为“五联征”。血小板计数（11.92±9.30）×109/L；间接胆红素（32.85±29.17）μmol/L、肌酐（110±69）μmol/L、乳酸脱氢酶（955±666）U/L。18例患者有ADAMTS13活性检查报告，其中酶活性＜10%者16例（88.9%）。24例患者（57.1%）予以大剂量血浆置换，存活17例（70.8%）；18例予以输注少量新鲜冰冻血浆或无血浆治疗，存活6例（33.3%）。PLASMIC评分6～7分33例；6～7分对预测ADAMTS13活性降低的准确性为88.9%。死亡组患者年龄更大，血肌酐水平更高（P均＜0.05）。结论：大部分TTP患者会表现为三联征，ADAMTS13活性检测对TTP的诊断具有一定提示作用，无条件时可使用PLASMIC评分进行早期拟诊。血浆置换治疗可明显降低TTP患者的死亡率，无条件者可输注少量新鲜冰冻血浆治疗。

英文摘要:

Abstract Objective: To analyze the clinical features, laboratory indicators, treatment, and outcomes of 42 patients with thrombotic thrombocytopenic purpura (TTP) in order to provide reference for clinical improvement. Methods: The basic information, clinical manifestations, laboratory examinations, treatment schemes, and outcomes of TTP patients diagnosed in multiple third-level hospitals in Hubei province were retrospectively analyzed. The patients were divided into survival group (23 cases) and death group (19 cases) according to the outcome. The differences in clinical features and different plasma treatments were compared between the two groups. The accuracy of predicting ADAMTS13 activity with PLASMIC score was calculated. Results: In this study, 42 patients were included, including 16 males (38.1%) and 26 females (61.9%); the median age of onset was 62 years, with an average age of (56.1±16.3) years. A total of 23 cases (54.8%) presented with the "triple syndrome", and 18 cases (42.9%) presented with the "pentasymptomatic syndrome". The platelet count was (11.92±9.30)×109/L; the indirect bilirubin was (32.85±29.17) μmol/L, creatinine was (110±69) μmol/L, and lactate dehydrogenase was (955±666) U/L. In total, 18 patients had ADAMTS13 activity reports, with 16 cases (88.9%) having enzyme activity <10%. A total of 24 cases (57.1%) received large-dose plasma exchange, with 17 cases (70.8%) surviving; Of 18 cases with little or no plasma exchange, 6 cases (33.3%) survived. The PLASMIC score was 6-7 in 33 cases. The accuracy of predicting a decrease in ADAMTS13 activity was 88.9% for patients with a score of 6-7. The age of the deceased patients was older and their serum creatinine levels were higher (P< 0.05). Conclusion: Most TTP patients present with the classic triad, and ADAMTS13 activity testing has a high diagnostic specificity for TTP. However, PLASMIC scoring can be used for early presumptive diagnosis when resources are limited. Plasma exchange therapy can significantly reduce the mortality rate of TTP patients, and a small amount of plasma can be administered to patients without resources.