Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.
Mastocytosis Centre, Odense University Hospital (MastOUH), Odense, Denmark.
Am J Hematol. 2016 Nov;91(11):1069-1075. doi: 10.1002/ajh.24490. Epub 2016 Aug 4.
In patients with systemic mastocytosis (SM), several aspects of morbidity remain poorly understood. We assessed the risk of solid cancers, cardiovascular disease, anaphylaxis, osteoporosis, and fractures in SM patients. Using Danish medical registries, we conducted a nationwide population-based cohort study including 687 adult (≥15 years) SM patients diagnosed during 1997-2012. A comparison cohort of 68,700 subjects from the general Danish population who were alive and without SM at the given SM subject's diagnosis were age- and gender-matched. Outcomes were a new diagnosis of solid cancer, venous thromboembolism (VTE), myocardial infarction (MI), stroke, anaphylaxis, osteoporosis, or fracture. For solid cancers the hazard ratio (HR) was 2.4 (95% confidence interval [CI] 1.9-2.8) with a 10-year absolute risk (AR) in the SM-cohort of 12.6% (95% CI 9.4-16.3). Specifically, we found a HR of 7.5 (95% CI 4.4-13.0) for melanoma and a HR of 2.5 (95% CI 1.7-3.5) for non-melanoma skin cancers (NMSCs). For VTE we found a HR of 1.9 (95% CI 1.2-3.0), with a 10-year AR of 3.9% (95% CI 2.3-6.1); for MI a nonsignificant increased HR of 1.4 (95% CI 0.9-2.3), with a 10-year AR of 1.8% (95% CI 0.9-3.2); and for stroke a HR of 1.6 (95% CI 1.1-2.3) with a 10-year AR of 4.6% (95% CI 2.8-6.9). The HR for anaphylaxis was 7.2 (95% CI 5.3-9.9), and the 10-year AR was 3.1% (95% CI 1.9-4.9). For osteoporosis the HR was 3.6 (95% CI 2.7-4.6) with a 10-year AR of 7.2% (95% CI 5.2-9.8). For fractures the HR was 1.2 (95% CI 0.9-1.6) and the 10-year AR was 5.9% (95% CI 3.9-8.4). SM patients are at increased risk of solid cancers - especially melanoma and NMSC-and cardiovascular disease. The risk of anaphylaxis and osteoporosis is clearly increased in SM, though absolute risk was low in this population-based study. The fracture-risk was only slightly increased. Am. J. Hematol. 91:1069-1075, 2016. © 2016 Wiley Periodicals, Inc.
在患有系统性肥大细胞增多症（SM）的患者中，其发病率的多个方面仍未得到很好的理解。我们评估了 SM 患者罹患实体癌、心血管疾病、过敏反应、骨质疏松症和骨折的风险。我们利用丹麦医疗登记系统，开展了一项全国性的基于人群的队列研究，纳入了 1997-2012 年间确诊的 687 名成年（≥15 岁）SM 患者。将来自一般丹麦人群的 68700 名年龄和性别相匹配、处于存活状态且无 SM 的受试者作为对照组。结局为新发实体癌、静脉血栓栓塞症（VTE）、心肌梗死（MI）、中风、过敏反应、骨质疏松症或骨折。对于实体癌，SM 队列的风险比（HR）为 2.4（95%置信区间[CI]为 1.9-2.8），10 年绝对风险（AR）为 12.6%（95%CI 为 9.4-16.3）。具体而言，我们发现黑色素瘤的 HR 为 7.5（95%CI 为 4.4-13.0），非黑色素瘤皮肤癌（NMSC）的 HR 为 2.5（95%CI 为 1.7-3.5）。对于 VTE，我们发现 HR 为 1.9（95%CI 为 1.2-3.0），10 年 AR 为 3.9%（95%CI 为 2.3-6.1）；对于 MI，未发现显著增加的 HR 为 1.4（95%CI 为 0.9-2.3），10 年 AR 为 1.8%（95%CI 为 0.9-3.2）；对于中风，HR 为 1.6（95%CI 为 1.1-2.3），10 年 AR 为 4.6%（95%CI 为 2.8-6.9）。过敏反应的 HR 为 7.2（95%CI 为 5.3-9.9），10 年 AR 为 3.1%（95%CI 为 1.9-4.9）。对于骨质疏松症，HR 为 3.6（95%CI 为 2.7-4.6），10 年 AR 为 7.2%（95%CI 为 5.2-9.8）。对于骨折，HR 为 1.2（95%CI 为 0.9-1.6），10 年 AR 为 5.9%（95%CI 为 3.9-8.4）。SM 患者罹患实体癌（尤其是黑色素瘤和 NMSC）和心血管疾病的风险增加。过敏反应和骨质疏松症的风险明显增加，尽管在这项基于人群的研究中，绝对风险较低。骨折风险仅略有增加。美国血液学杂志 91:1069-1075，2016 年。©2016 年威利期刊公司。
