意大利肥大细胞增多症登记处：基于医院登记处的 6 年经验。

The Italian Mastocytosis Registry: 6-year experience from a hospital-based registry.

机构信息

Hematology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.

Hematology Department, James Cook University Hospital, South Tees Trust (NHS), Middlesbrough, UK.

出版信息

Future Oncol. 2018 Nov;14(26):2713-2723. doi: 10.2217/fon-2018-0291. Epub 2018 Sep 12.

DOI:10.2217/fon-2018-0291

PMID:30207489

Abstract

AIM

We collected 'real-life' data on the management of patients with mastocytosis in the Italian Mastocytosis Registry.

METHODS

Six hundred patients diagnosed with mastocytosis between 1974 and 2014 were included from 19 centers.

RESULTS

Among adults (n = 401); 156 (38.9%) patients were diagnosed with systemic mastocytosis. In 212 adults, no bone marrow studies were performed resulting in a provisional diagnosis of mastocytosis of the skin. This diagnosis was most frequently established in nonhematologic centers. In total, 182/184 pediatric patients had cutaneous mastocytosis. We confirmed that in the most patients with systemic mastocytosis, serum tryptase levels were >20 ng/ml and KIT D816V was detectable.

CONCLUSION

The Italian Mastocytosis Registry revealed some center-specific approaches for diagnosis and therapy. Epidemiological evidence on this condition is provided.

摘要

目的

我们从意大利肥大细胞增多症登记处收集了肥大细胞增多症患者管理的“真实数据”。

方法

从 19 个中心共纳入了 1974 年至 2014 年间诊断为肥大细胞增多症的 600 名患者。

结果

在成年患者中（n=401），156 名（38.9%）患者被诊断为系统性肥大细胞增多症。在 212 名成年患者中，未进行骨髓研究，导致临时诊断为皮肤肥大细胞增多症。该诊断最常在非血液学中心建立。在总共 182/184 名儿科患者中，肥大细胞均存在于皮肤中。我们证实，在大多数系统性肥大细胞增多症患者中，血清胰蛋白酶水平>20ng/ml，并且可以检测到 KIT D816V。