Mori Francesca, Crisafulli Giuseppe, Bianchi Annamaria, Bottau Paolo, Caimmi Silvia, Franceschini Fabrizio, Liotti Lucia, Paglialunga Claudia, Saretta Francesca, Caffarelli Carlo
Allergologia, Dipartimento di Pediatria, Ospedale Anna Meyer, 50132 Firenze, Italy.
UOC di Pediatria, Dipartimento Materno-Infantile, Università di Messina, 98168 Messina, Italy.
J Clin Med. 2022 Jun 1;11(11):3153. doi: 10.3390/jcm11113153.
Mastocytosis, a heterogeneous mastcell disease, include three different entities: cutaneous mastocytosis, systemic mastocytosis (SM) and mast-cell sarcoma. Tryptase levels can differentiate cutaneous mastocytosis from SM. In mastocytosis, quick onset drug hypersensitivity reactions (DHRs) that are facilitated by mastcell mediators, are investigated in adults. Due to the limited number of children with mastcell disease and increased serum tryptase levels, the role of drugs in this age group is less studied. In this review, we critically assessed relevant papers related with immediate DHRs in children with mastocytosis and discuss practical issues of the management. In childhood mastocytosis, anaphylaxis is frequently idiopathic, and elevated level of basal tryptase, and high burden of disease may increase the risk. Among drugs, antibiotics, NSAIDs and opioids can potentially induce anaphylaxis, anyway avoidance should be recommended only in case of previous reactions. Moreover, vaccinations are not contraindicated in patients with mastocytosis. The risk of severe systemic reactions after drugs intake seems to be extremely low and in general lower in children than in adults. Anyway, studies on this topic especially focusing on children, are missing to state final recommendations.
肥大细胞增多症是一种异质性肥大细胞疾病，包括三种不同类型：皮肤肥大细胞增多症、系统性肥大细胞增多症（SM）和肥大细胞肉瘤。类胰蛋白酶水平可用于区分皮肤肥大细胞增多症和系统性肥大细胞增多症。在肥大细胞增多症中，研究了成人中由肥大细胞介质促成的速发型药物超敏反应（DHRs）。由于患有肥大细胞疾病的儿童数量有限且血清类胰蛋白酶水平升高，该年龄组中药物的作用研究较少。在本综述中，我们批判性地评估了与肥大细胞增多症患儿速发型DHRs相关的论文，并讨论了管理中的实际问题。在儿童肥大细胞增多症中，过敏反应通常是特发性的，基础类胰蛋白酶水平升高和疾病负担加重可能会增加风险。在药物中，抗生素、非甾体抗炎药和阿片类药物可能会诱发过敏反应，无论如何，仅在既往有反应的情况下才建议避免使用。此外，肥大细胞增多症患者接种疫苗并无禁忌。用药后发生严重全身反应的风险似乎极低，总体而言儿童低于成人。无论如何，缺乏专门针对儿童的该主题研究以给出最终建议。
