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出血评估工具:遗传性出血性疾病诊断和预后的局限性与优势

Bleeding Assessment Tools: Limits and Advantages for the Diagnosis and Prognosis of Inherited Bleeding Disorders.

作者信息

Tosetto Alberto

机构信息

Hematology Department, S. Bortolo Hospital, Vicenza, Italy.

出版信息

Semin Thromb Hemost. 2016 Jul;42(5):463-70. doi: 10.1055/s-0036-1571313. Epub 2016 Apr 20.

Abstract

Bleeding assessment tools were first developed essentially as research tools, for the quantification of bleeding symptoms and the study of phenotype/genotype correlations. Interestingly, these tools have been proven useful also for clinicians diagnosing and treating bleeding disorders. The main advantage of these tools is the standardization of the diagnostic process, allowing the introduction of criteria with known specificity and sensitivity for the diagnosis of the most common mild bleeding disorders, particularly von Willebrand disease. This is important also for a rational approach to the laboratory diagnosis because for many mild bleeding disorders, a complex laboratory workup is required. Bleeding assessment tools should always be complemented by ancillary coagulation screening tests to exclude the presence of a bleeding disorder, however. Finally, bleeding severity assessed by such tools has been shown to correlate with the long-term probability of bleeding. Therefore, the bleeding assessment could become an important marker of disease severity.

摘要

出血评估工具最初主要是作为研究工具开发的,用于量化出血症状以及研究表型/基因型的相关性。有趣的是,这些工具已被证明对诊断和治疗出血性疾病的临床医生也很有用。这些工具的主要优点是诊断过程的标准化,允许引入对最常见的轻度出血性疾病,特别是血管性血友病诊断具有已知特异性和敏感性的标准。这对于合理的实验室诊断方法也很重要,因为对于许多轻度出血性疾病,需要进行复杂的实验室检查。然而,出血评估工具始终应辅以辅助凝血筛查试验,以排除出血性疾病的存在。最后,通过这些工具评估的出血严重程度已被证明与出血的长期可能性相关。因此,出血评估可能成为疾病严重程度的重要指标。

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