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Multidisciplinary Challenges in Mastocytosis and How to Address with Personalized Medicine Approaches

1
Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, 1090 Vienna, Austria
2
Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, 1090 Vienna, Austria
3
Division of Allergy and Clinical Immunology, University of Michigan, Ann Arbor, MI 48106, USA
4
III. Medizinische Klinik, Universitätsmedizin Mannheim, 68167 Mannheim, Germany
5
Department of Hematological Biology, Pitié-Salpêtrière Hospital, Pierre et Marie Curie University (UPMC), 75005 Paris, France
6
Division of Allergy and Clinical Immunology, University of Salerno, 84131 Salerno, Italy
*
Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2019, 20(12), 2976; https://doi.org/10.3390/ijms20122976
Received: 26 March 2019 / Revised: 5 June 2019 / Accepted: 16 June 2019 / Published: 18 June 2019
(This article belongs to the Special Issue Mast Cells in Health and Disease)
Mastocytosis is a hematopoietic neoplasm defined by abnormal expansion and focal accumulation of clonal tissue mast cells in various organ-systems. The disease exhibits a complex pathology and an equally complex clinical behavior. The classification of the World Health Organization (WHO) divides mastocytosis into cutaneous forms, systemic variants, and localized mast cell tumors. In >80% of patients with systemic mastocytosis (SM), a somatic point mutation in KIT at codon 816 is found. Whereas patients with indolent forms of the disease have a normal or near-normal life expectancy, patients with advanced mast cell neoplasms, including aggressive SM and mast cell leukemia, have a poor prognosis with short survival times. In a majority of these patients, multiple somatic mutations and/or an associated hematologic neoplasm, such as a myeloid leukemia, may be detected. Independent of the category of mastocytosis and the serum tryptase level, patients may suffer from mediator-related symptoms and/or osteopathy. Depending on the presence of co-morbidities, the symptomatology in such patients may be mild, severe or even life-threatening. Most relevant co-morbidities in such patients are IgE-dependent allergies, psychiatric, psychological or mental problems, and vitamin D deficiency. The diagnosis and management of mastocytosis is an emerging challenge in clinical practice and requires vast knowledge, a multidisciplinary approach, and personalized medicine procedures. In this article, the current knowledge about mastocytosis is reviewed with special emphasis on the multidisciplinary aspects of the disease and related challenges in daily practice. View Full-Text
Keywords: mast cells; KIT D816V; tryptase; IgE; allergy; MCAS; personalized medicine mast cells; KIT D816V; tryptase; IgE; allergy; MCAS; personalized medicine
MDPI and ACS Style

Valent, P.; Akin, C.; Gleixner, K.V.; Sperr, W.R.; Reiter, A.; Arock, M.; Triggiani, M. Multidisciplinary Challenges in Mastocytosis and How to Address with Personalized Medicine Approaches. Int. J. Mol. Sci. 2019, 20, 2976.

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