血栓性血小板减少性紫癜的实验室调查和诊断。

IF 6.6 2区医学 Q1 MEDICAL LABORATORY TECHNOLOGY

Critical reviews in clinical laboratory sciences Pub Date : 2023-12-01 Epub Date: 2023-07-14 DOI:10.1080/10408363.2023.2232039

Konstantinos Dimopoulos, Armando Tripodi, Jens P Goetze

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引用次数: 0

摘要

血栓性血小板减少性紫癜(TTP)是一种罕见且潜在致命的疾病，快速诊断对患者预后至关重要。肝酶ADAMTS13活性不足(< 10%)是TTP的病理生理标志，测定酶活性可以建立TTP的高精度诊断。因此，结合临床病史，对疑似TTP病例进行适当的实验室评估对于诊断和治疗至关重要。在这里，我们回顾了现有的实验室测试，可以帮助临床医生建立TTP的诊断，特别关注ADAMTS13的测定，包括抗原和活性的测量，以及对ADAMTS13的自身抗体的检测。

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Laboratory investigation and diagnosis of thrombotic thrombocytopenic purpura.

Thrombotic thrombocytopenic purpura (TTP) is a rare and potentially fatal disease for which rapid diagnosis is crucial for patient outcomes. Deficient activity (< 10%) of the liver enzyme, ADAMTS13, is the pathophysiological hallmark of TTP, and measurement of the enzyme activity can establish the diagnosis of TTP with high accuracy. Thus, along with the clinical history, appropriate laboratory assessment of a suspected case of TTP is essential for diagnosis and treatment. Here, we present a review of the available laboratory tests that can assist clinicians in establishing the diagnosis of TTP, with special focus on ADAMTS13 assays, including the measurement of the antigen and activity, and detection of autoantibodies to ADAMTS13.

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来源期刊

Critical reviews in clinical laboratory sciences 医学-医学实验技术

CiteScore

20.00

自引率

0.00%

发文量

审稿时长

>12 weeks

期刊介绍： Critical Reviews in Clinical Laboratory Sciences publishes comprehensive and high quality review articles in all areas of clinical laboratory science, including clinical biochemistry, hematology, microbiology, pathology, transfusion medicine, genetics, immunology and molecular diagnostics. The reviews critically evaluate the status of current issues in the selected areas, with a focus on clinical laboratory diagnostics and latest advances. The adjective “critical” implies a balanced synthesis of results and conclusions that are frequently contradictory and controversial.