背景:肥大细胞疾病包括肥大细胞增多症和肥大细胞活化综合征,其中一些已被证明涉及肥大细胞的克隆缺陷,导致异常的细胞增殖或活化。已经发表了许多关于肥大细胞增多症的临床研究,但在美国尚未发现以人群为基础的全面患者调查.美国很少有肥大细胞病专业中心,非专科医生对这些肥大细胞疾病的认识有限。因此,缺乏有关这些患者总体估计人群经历的信息.
目的:为了确定肥大细胞增多症患者的经历和看法,肥大细胞活化综合征,和相关的疾病,肥大细胞学会(TMS),基于美国的患者倡导,研究,和教育组织,对其成员和其他已知或怀疑是该患者群体的一部分的人进行了调查。
方法:通过治疗这些患者的诊所和TMS的时事通讯公布了一项基于网络的调查,Web站点,和在线博客。提供了问卷的在线和纸质副本,以及所需的同意声明。
结果:第一个结果为420名患者。这些结果包括人口统计,诊断,症状,过敏,引起肥大细胞症状的因素,和疾病的影响。
结论:患有肥大细胞增多症和肥大细胞活化综合征的患者为临床专家,合作者,和其他患者的信息,使他们能够探索和加深他们对应对这些疾病的人的经验和看法的理解。
BACKGROUND: Mast cell diseases include mastocytosis and mast cell activation syndromes, some of which have been shown to involve clonal defects in mast cells that result in abnormal cellular proliferation or activation. Numerous clinical studies of mastocytosis have been published, but no population-based comprehensive surveys of patients in the United States have been identified. Few mast cell disease specialty centers exist in the United States, and awareness of these mast cell disorders is limited among nonspecialists. Accordingly, information concerning the experiences of the overall estimated population of these patients has been lacking.
OBJECTIVE: To identify the experiences and perceptions of patients with mastocytosis, mast cell activation syndromes, and related disorders, The Mastocytosis Society (TMS), a US based patient advocacy, research, and education organization, conducted a survey of its members and other people known or suspected to be part of this patient population.
METHODS: A Web-based survey was publicized through clinics that treat these patients and through TMS\'s newsletter, Web site, and online blogs. Both online and paper copies of the questionnaire were provided, together with required statements of consent.
RESULTS: The first results are presented for 420 patients. These results include demographics, diagnoses, symptoms, allergies, provoking factors of mast cell symptoms, and disease impact.
CONCLUSIONS: Patients with mastocytosis and mast cell activation syndromes have provided clinical specialists, collaborators, and other patients with information to enable them to explore and deepen their understanding of the experiences and perceptions of people coping with these disorders.