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Review

Epigenetic Changes in Neoplastic Mast Cells and Potential Impact in Mastocytosis

1
Department of Molecular Genetics and Epigenetics, Nofer Institute of Occupational Medicine, 91-348 Lodz, Poland
2
Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, 1090 Vienna, Austria
3
Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, 1090 Vienna, Austria
4
Department of Hematological Biology, Pitié-Sapêtrière Hospital, Sorbonne University, 75013 Paris, France
5
Division of Immunology and Allergy, Department of Medicine, Karolinska Institutet and Karolinska University Hospital, SE-171 76 Stockholm, Sweden
6
Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, 80-211 Gdansk, Poland
7
Invicta Fertility and Reproductive Center, Molecular Laboratory, Polna 64, 81-740 Sopot, Poland
8
Department of Allergology, Medical University of Gdansk, 80-211 Gdansk, Poland
*
Authors to whom correspondence should be addressed.
Academic Editor: Gianni Marone
Int. J. Mol. Sci. 2021, 22(6), 2964; https://doi.org/10.3390/ijms22062964
Received: 18 February 2021 / Revised: 10 March 2021 / Accepted: 12 March 2021 / Published: 15 March 2021
Systemic mastocytosis (SM) is a hematologic neoplasm with abnormal accumulation of mast cells in various organ systems such as the bone marrow, other visceral organs and skin. So far, only little is known about epigenetic changes contributing to the pathogenesis of SM. In the current article, we provide an overview of epigenetic changes that may occur and be relevant to mastocytosis, including mutations in genes involved in epigenetic processes, such as TET2, DNMT3A and ASXL1, and global and gene-specific methylation patterns in neoplastic cells. Moreover, we discuss methylation-specific pathways and other epigenetic events that may trigger disease progression in mast cell neoplasms. Finally, we discuss epigenetic targets and the effects of epigenetic drugs, such as demethylating agents and BET-targeting drugs, on growth and viability of neoplastic mast cells. The definitive impact of these targets and the efficacy of epigenetic therapies in advanced SM need to be explored in future preclinical studies and clinical trials. View Full-Text
Keywords: mastocytosis; epigenetics; DNA methylation; demethylating agents; DNMT3A; TET2; microRNA mastocytosis; epigenetics; DNA methylation; demethylating agents; DNMT3A; TET2; microRNA
MDPI and ACS Style

Reszka, E.; Jabłońska, E.; Wieczorek, E.; Valent, P.; Arock, M.; Nilsson, G.; Nedoszytko, B.; Niedoszytko, M. Epigenetic Changes in Neoplastic Mast Cells and Potential Impact in Mastocytosis. Int. J. Mol. Sci. 2021, 22, 2964. https://doi.org/10.3390/ijms22062964

AMA Style

Reszka E, Jabłońska E, Wieczorek E, Valent P, Arock M, Nilsson G, Nedoszytko B, Niedoszytko M. Epigenetic Changes in Neoplastic Mast Cells and Potential Impact in Mastocytosis. International Journal of Molecular Sciences. 2021; 22(6):2964. https://doi.org/10.3390/ijms22062964

Chicago/Turabian Style

Reszka, Edyta, Ewa Jabłońska, Edyta Wieczorek, Peter Valent, Michel Arock, Gunnar Nilsson, Bogusław Nedoszytko, and Marek Niedoszytko. 2021. "Epigenetic Changes in Neoplastic Mast Cells and Potential Impact in Mastocytosis" International Journal of Molecular Sciences 22, no. 6: 2964. https://doi.org/10.3390/ijms22062964

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