背景:超敏反应(HR)在肥大细胞增多症中很常见。然而,对触发因素和危险因素知之甚少。欧洲肥大细胞增多症能力网络(ECNM)的注册能够在更大的肥大细胞增多症患者队列中进行可靠的研究。我们评估了患病率,ECNM注册中成人肥大细胞增多症的HR触发因素和危险因素。
方法:收集27个ECNM中心的数据。我们分析了潜在的触发因素(膜翅目毒液,食物,药物,吸入剂和其他)以及诊断和随访期间的危险因素。研究组由2485名患有肥大细胞增多症的成年人组成,1379名女性(55.5%)和1106名男性(44.5%)。中位年龄为48.2岁(范围18-91岁)。
结果:九百四十八例患者(38.1%)报告了一个或多个HR。最常见的触发因素是皮肤肥大细胞增多症(CM)和惰性系统性肥大细胞增多症(ISM)的膜翅目毒液,而在高级SM(advSM)中,最常见的激发者是毒品,包括非甾体抗炎药和青霉素。在多变量分析中,类胰蛋白酶水平<90ng/mL,骨髓活检切片肥大细胞浸润<15%,和ISM的诊断被确定为HR的独立危险因素。对于药物诱导的HR,主要的危险因素是advSM和高类胰蛋白酶水平。在4年的随访中,所有患者中有4.8%观察到新的反应。
结论:HR主要由CM和ISM患者的膜翅目毒液和advSM患者的药物触发。类胰蛋白酶水平<90ng/mL,肥大细胞骨髓浸润<15%,和WHO类别ISM是人力资源的预测因子。在4年内,所有患者中有4.8%出现新的HR。
BACKGROUND: Hypersensitivity reactions (HR) are common in
mastocytosis. However, little is known about triggers and risk factors. The registry of the European Competence Network on
Mastocytosis (ECNM) enables reliable studies in a larger cohort of
mastocytosis patients. We assessed prevalence, triggers and risk factors of HR in adults with
mastocytosis in the ECNM registry.
METHODS: Data were collected in 27 ECNM centers. We analyzed potential triggers (Hymenoptera venoms, food, drug, inhalant and others) and risk factors at diagnosis and during follow-up. The study group consisted of 2485 adults with mastocytosis, 1379 women (55.5%) and 1106 men (44.5%). Median age was 48.2 years (range 18-91 years).
RESULTS: Nine hundred and forty eight patients (38.1%) reported one or more HR`. Most common triggers were Hymenoptera venoms in cutaneous
mastocytosis (CM) and indolent systemic
mastocytosis (ISM), whereas in advanced SM (advSM), most common elicitors were drugs, including nonsteroidal anti-inflammatory agents and penicillin. In multivariate analyses, tryptase level < 90 ng/mL, <15% infiltration by mast cells in bone marrow biopsy-sections, and diagnosis of ISM were identified as independent risk factors for HR. For drug-induced HR, prominent risk factors were advSM and high tryptase levels. New reactions were observed in 4.8% of all patients during 4 years follow-up.
CONCLUSIONS: HR are mainly triggered by Hymenoptera venoms in patients with CM and ISM and by drugs in patients with advSM. Tryptase levels <90 ng/mL, mast cell bone marrow infiltration <15%, and WHO category ISM are predictors of HR. New HR occur in 4.8% of all patients within 4 years.