Onnes Merel C, Alheraky Abdulrazzaq, Nawijn Martijn C, Sluijter Tim E, Mulder André B, Arends Suzanne, Oude Elberink Hanneke N G
Department of Internal Medicine Division of Allergology University Medical Center Groningen University of Groningen Groningen The Netherlands.
Department of Pathology and Medical Biology University Medical Center Groningen University of Groningen Groningen The Netherlands.
Clin Transl Allergy. 2022 Sep 7;12(9):e12174. doi: 10.1002/clt2.12174. eCollection 2022 Sep.
Clonal mast cell disease (CMD) is an underlying aggravating condition in wasp venom allergy (WVA) which requires a different treatment strategy. CMD is increasingly recognized in patients with normal basal serum tryptase (bsT). However, methods to identify at risk patients have not yet been assessed in large cohorts of WVA patients with normal bsT.
This retrospective study evaluated the reliability of the REMA score in detecting CMD in a cohort of grade IV WVA patients with normal bsT and assessed the added value of other clinical parameters, D816V mutation analysis in peripheral blood (PB) and the diagnosis of hereditary alpha tryptasemia (HAT). All patients had a conclusive bone marrow evaluation that demonstrated or excluded underlying CMD.
In total 35 CMD and 96 non-CMD patients were included. REMA score had a sensitivity of 72% (95% CI 56%-88%) and specificity of 79% (95% CI 70%-87%) in this cohort. Loss of consciousness during systemic reaction and bsT between 6.3 and 11.4 ng/ml were additional parameters independently associated with CMD. Sensitivity of in PB was relatively low, 56% (95% CI 36%-75%), but had added value as screening method in patients with a low REMA score due to 100% specificity.
The REMA score is a relatively reliable method to detect patients at risk of CMD among WVA patients with normal bsT. mutation analysis in PB could serve as additional screening method in patients with low REMA scores.
克隆性肥大细胞疾病（CMD）是黄蜂毒液过敏（WVA）的一种潜在加重情况，需要不同的治疗策略。CMD在基础血清类胰蛋白酶（bsT）正常的患者中越来越受到认可。然而，尚未在大量bsT正常的WVA患者队列中评估识别高危患者的方法。
这项回顾性研究评估了REMA评分在检测bsT正常的IV级WVA患者队列中CMD的可靠性，并评估了其他临床参数、外周血（PB）中D816V突变分析以及遗传性α类胰蛋白酶血症（HAT）诊断的附加价值。所有患者均进行了结论性的骨髓评估，以证实或排除潜在的CMD。
总共纳入了35例CMD患者和96例非CMD患者。在该队列中，REMA评分的敏感性为72%（95%CI 56%-88%），特异性为79%（95%CI 70%-87%）。全身反应期间的意识丧失和bsT在6.3至11.4 ng/ml之间是与CMD独立相关的其他参数。PB中 的敏感性相对较低，为56%（95%CI 36%-75%），但作为REMA评分低的患者的筛查方法具有附加价值，因为其特异性为100%。
REMA评分是检测bsT正常的WVA患者中CMD高危患者的相对可靠方法。PB中的 突变分析可作为REMA评分低的患者的附加筛查方法。
