Kibsgaard Line, Deleuran Mette, Flohr Carsten, Langan Sinéad, Braae Olesen Anne, Vestergaard Christian
Department of Dermatology, Aarhus University Hospital, Aarhus, Denmark.
Unit for Population-based Dermatology Research, St John's Institute of Dermatology, Guy's & St Thomas' NHS Foundation Trust and Kings College London, London, United Kingdom.
Int J Womens Dermatol. 2020 Jun 1;6(4):294-300. doi: 10.1016/j.ijwd.2020.05.013. eCollection 2020 Sep.
There are limited estimates of the incidence rates (IRs) of mastocytosis, and only a few studies have addressed the long-term consequences of living with these diagnoses. Previous reports have shown that systemic mastocytosis is associated with leukemic transformations and an increased risk of death as opposed to cutaneous mastocytosis (CM) and indolent systemic mastocytosis (ISM), which have benign diagnoses with life expectancy rates similar to those of the background population.
This study aimed to analyze the incidence and mortality of mastocytosis.
A population-based matched cohort study of patients with mastocytosis between 1 January 1, 1977 and 31 December 31, 2014 was identified from the Danish National Health Registries. IRs of CM, ISM, and pediatric mastocytosis were highlighted. Survival estimates were compared with those of a healthy background population, using a Cox proportional hazard model.
A total of 1461 patients with mastocytosis were identified. The annual IR of overall mastocytosis was 1.1 per 100,000 person years (95% confidence interval [CI], 1.0-1.2). Among children, the IR was 1.8 per 100,000 person years (95% CI, 1.6-2.1). The prevalence of any comorbidity was twice as high among patients with mastocytosis compared with the population without mastocytosis (odds ratio: 2.1; 95% CI, 1.8-2.5). The Charlson Comorbidity Index-adjusted mortality among adult patients with mastocytosis was HR 1.2 (95% CI, 0.8-1.9), HR 1.9 (95% CI 1.4-2.5), and HR 4.2 (95%, CI 1.9-9.4), respectively.
Based on an entire nation, with free health care at the point of access, we estimated an annual IR of mastocytosis and its subgroups. We discovered that patients with ISM had an increased risk of death compared with the general population. Our data supported the overall benign nature of CM diagnosed after age 2 years.
肥大细胞增多症的发病率估计有限，仅有少数研究探讨了患有这些疾病诊断的长期后果。先前的报告表明，系统性肥大细胞增多症与白血病转化及死亡风险增加有关，而皮肤肥大细胞增多症（CM）和惰性系统性肥大细胞增多症（ISM）则具有良性诊断，预期寿命与背景人群相似。
本研究旨在分析肥大细胞增多症的发病率和死亡率。
从丹麦国家卫生注册中心确定了一项基于人群的匹配队列研究，研究对象为1977年1月1日至2014年12月31日期间的肥大细胞增多症患者。突出了CM、ISM和儿童肥大细胞增多症的发病率。使用Cox比例风险模型将生存估计值与健康背景人群进行比较。
共识别出1461例肥大细胞增多症患者。总体肥大细胞增多症的年发病率为每10万人年1.1例（95%置信区间[CI]，1.0 - 1.2）。在儿童中，发病率为每10万人年1.8例（95%CI，1.6 - 2.1）。与无肥大细胞增多症的人群相比，肥大细胞增多症患者中任何合并症的患病率高出两倍（比值比：2.1；95%CI，1.8 - 2.5）。成人肥大细胞增多症患者经Charlson合并症指数调整后的死亡率分别为HR 1.2（95%CI，0.8 - 1.9）、HR 1.9（95%CI 1.4 - 2.5）和HR 4.2（95%，CI 1.9 - 9.4）。
基于一个全民享有免费医疗服务的国家，我们估计了肥大细胞增多症及其亚组的年发病率。我们发现，与普通人群相比，ISM患者的死亡风险增加。我们的数据支持2岁后诊断的CM总体具有良性性质。
