Original Article
The Mastocytosis Society Survey on Mast Cell Disorders: Patient Experiences and Perceptions

https://doi.org/10.1016/j.jaip.2013.09.004Get rights and content
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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.

Conclusion

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.

Key words

Survey
Mast cell disorders
The Mastocytosis Society
Mast cell activation syndrome
Urticaria pigmentosa
Anaphylaxis
Food intolerance
Insect venom
Trigger
Allergy

Abbreviations used

AFIRMM
Association Française pour les Initiatives de Recherche sur le Mastocytes et les Mastocytoses
CM
Cutaneous mastocytosis
IA
Idiopathic anaphylaxis
Ig
Immunoglobulin
MC
Mast cell
MCA
Mast cell activation
MCAD
Mast cell activation disorder
MCAS
Mast cell activation syndrome
MCD
Mast cell disorder
RAST
Radioallergosorbent test
REMA
Red Española de Mastocitosis
SM
Systemic mastocytosis
TMEP
Telangiectasia macularis eruptiva perstans
TMS
The Mastocytosis Society, Inc
UP
Urticaria pigmentosa

Cited by (0)

This work was primarily conducted through volunteer efforts of The Mastocytosis Society (TMS) Research Committee and Board of Directors, with scientific and medical questions resolved by those with relevant background and expertise. Minimal costs of postage and online survey and database hosting were supported by TMS. The authors obtained no funding for activities related to this report. Grants from TMS not directly related to conducting, analyzing, or reporting of this patient survey were received by C. Akin and M. Castells in the years before the survey and by P. Valent after survey closure. M. Castells, P. Valent, and C. Akin are TMS Medical Advisory Board members.

Conflicts of interest: S. Jennings, N. Russell, and V. Slee have received travel support from The Mastocytosis Society (TMS). M. Castells has received a grant from TMS; has consultant arrangements with Merck, Novartis, and Genentech; and is employed by Brigham and Women's Hospital. P. Valent has received grants from TMS and Novartis, has consultant arrangements with Novartis, and has received a speaker's honorarium from Novartis. C. Akin has consultant arrangements with Novartis and Best Doctors; is employed by Brigham and Women's Hospital; has received grants from TMS; has received travel reimbursement and an honorarium from the Western Society of Allergy; has received travel reimbursement and a waived registration fee from the European Academy of Allergy and Clinical Immunology; has received a waived registration fee from the American Academy of Allergy, Asthma and Immunology; has a patent with the National Institutes of Health for an LAD2 mast cell line; and has received royalties from UpToDate. The rest of the authors declare that they have no relevant conflicts of interest.