Suleiman Mathieu N, Brueckl Valeska, Fechner Jörg, Kaemmerer Ann-Sophie, Wilk Florian, Weyand Michael, Harig Frank
Department of Cardiac Surgery, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg, 91054 Erlangen, Germany.
Department of Haematology and Oncology, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg, 91054 Erlangen, Germany.
J Clin Med. 2023 Feb 1;12(3):1156. doi: 10.3390/jcm12031156.
(1) Background: Systemic mastocytosis is a rare, non-curable disease with potential life-threatening complications in patients receiving cardiac surgery. (2) Methods: This systematic review of the literature was prompted by the case of a life-threatening anaphylactic reaction during cardiac surgery related to systemic mastocytosis. The search of all types of studies, using several databases (Pubmed, Scopus and Web of Science), was conducted through September 2022 to identify the relevant studies. (3) Results: Twelve studies were included describing cases of patients undergoing cardiac surgery who were diagnosed with systemic mastocytosis. An adverse effect, namely anaphylaxis, has happened in three cases. Different strategies of premedication, intraoperative and postoperative management were used. In our case, the patient was admitted for elective biological aortic valve replacement due to severe aortic stenosis. Intraoperatively, the patient developed an anaphylactic shock during the administration of protamine after separation from the cardiopulmonary bypass. This anaphylaxis reaction was a complication of the pre-existing systemic mastocytosis and could be successfully managed by the administration of epinephrine, antihistamines and corticosteroids. (4) Conclusions: This systematic literature search and case report highlight the importance of careful preoperative planning, as well as coordination between cardiac surgeons, anesthesiologists and hemato-oncological specialists, in patients with rare but complication-prone diseases such as systemic mastocytosis.
(1) 背景：系统性肥大细胞增多症是一种罕见的、无法治愈的疾病，接受心脏手术的患者可能会出现危及生命的并发症。(2) 方法：本次文献系统综述是由一例心脏手术期间与系统性肥大细胞增多症相关的危及生命的过敏反应病例引发的。通过搜索多个数据库（PubMed、Scopus和Web of Science），检索截至2022年9月的各类研究，以确定相关研究。(3) 结果：纳入了12项描述接受心脏手术且被诊断为系统性肥大细胞增多症患者病例的研究。有3例出现了不良反应，即过敏反应。采用了不同的术前用药、术中和术后管理策略。在我们的病例中，患者因严重主动脉瓣狭窄入院接受择期生物主动脉瓣置换术。术中，患者在体外循环分离后注射鱼精蛋白时发生过敏性休克。这种过敏反应是既往存在的系统性肥大细胞增多症的并发症，通过给予肾上腺素、抗组胺药和糖皮质激素可成功处理。(4) 结论：本次系统文献检索和病例报告强调了对于系统性肥大细胞增多症等罕见但易出现并发症的疾病患者，术前仔细规划以及心脏外科医生、麻醉医生和血液肿瘤专科医生之间协调配合的重要性。
