PDF(Pubmed)
Abstract：
Mast cell (MC) activation syndromes (MCAS) are conditions defined by recurrent episodes of severe systemic anaphylaxis or similar systemic events triggered by MC-derived mediators that can be measured in biological fluids. Since some symptoms of MC activation may occur due to other, non-MC etiologies and lead to confusion over diagnosis, it is of crucial importance to document the involvement of MC and their products in the patients´ symptomatology.
The most specific and generally accepted marker of severe systemic MC activation is an event-related, transient increase in the serum tryptase level over the individual baseline of the affected individual. However, baseline concentrations of serum tryptase vary among donors, depending on the genetic background, age, kidney function, and underlying disease. As a result, it is of critical importance to provide a flexible equation that defines the diagnostic increase in tryptase qualifying as MCAS criterion in all patients, all situations, and all ranges of baseline serum tryptase. In 2012, the consensus group proposed the 120% + 2 ng/ml formula, which covers the great majority of groups, including cases with low, normal, or elevated basal serum tryptase level. This formula has been validated in subsequent studies and has proven to be a robust and consistent diagnostic criterion of MCAS. The present article is discussing the impact of this formula and possible limitations as well as alternative markers and mediators that may be indicative of MCAS.
The most specific and generally accepted marker of severe systemic MC activation is an event-related, transient increase in the serum tryptase level over the individual baseline of the affected individual. However, baseline concentrations of serum tryptase vary among donors, depending on the genetic background, age, kidney function, and underlying disease. As a result, it is of critical importance to provide a flexible equation that defines the diagnostic increase in tryptase qualifying as MCAS criterion in all patients, all situations, and all ranges of baseline serum tryptase. In 2012, the consensus group proposed the 120% + 2 ng/ml formula, which covers the great majority of groups, including cases with low, normal, or elevated basal serum tryptase level. This formula has been validated in subsequent studies and has proven to be a robust and consistent diagnostic criterion of MCAS. The present article is discussing the impact of this formula and possible limitations as well as alternative markers and mediators that may be indicative of MCAS.
摘要：
目的：肥大细胞（MC）激活综合征（MCAS）是由可在生物体液中测量的MC衍生介质触发的严重全身过敏反应或类似全身事件的反复发作定义的病症。由于MC激活的某些症状可能由于其他原因而发生，非MC病因，导致诊断混乱，记录MC及其产品在患者症状学中的参与至关重要。
结果：严重全身性MC激活的最特异性和普遍接受的标志物是与事件相关的，血清类胰蛋白酶水平在受影响个体的个体基线上短暂增加。然而,血清类胰蛋白酶的基线浓度因供体而异，根据遗传背景，年龄,肾功能,和潜在的疾病。因此,提供一个灵活的公式来定义所有患者中符合MCAS标准的类胰蛋白酶的诊断增加是至关重要的。所有情况,和所有范围的基线血清类胰蛋白酶。2012年，共识小组提出了120%+2ng/ml的公式，涵盖了绝大多数群体，包括低的病例，正常,或基础血清类胰蛋白酶水平升高。该公式已在随后的研究中得到验证，并已被证明是MCAS的稳健且一致的诊断标准。本文正在讨论该公式的影响和可能的局限性以及可能指示MCAS的替代标记和介体。
结果：严重全身性MC激活的最特异性和普遍接受的标志物是与事件相关的，血清类胰蛋白酶水平在受影响个体的个体基线上短暂增加。然而,血清类胰蛋白酶的基线浓度因供体而异，根据遗传背景，年龄,肾功能,和潜在的疾病。因此,提供一个灵活的公式来定义所有患者中符合MCAS标准的类胰蛋白酶的诊断增加是至关重要的。所有情况,和所有范围的基线血清类胰蛋白酶。2012年，共识小组提出了120%+2ng/ml的公式，涵盖了绝大多数群体，包括低的病例，正常,或基础血清类胰蛋白酶水平升高。该公式已在随后的研究中得到验证，并已被证明是MCAS的稳健且一致的诊断标准。本文正在讨论该公式的影响和可能的局限性以及可能指示MCAS的替代标记和介体。
