Los mastocitos son células inmunitarias multifuncionales:




De esta manera, el mecanismo fisiopatológico por el cual aparece la mastocitosis es el siguiente:2



1. Theoharides TC, Valent P, Akin C. Mast Cells, Mastocytosis, and Related Disorders. N Engl J Med. 2015 9;373(2):163-72. 2. Orfao A, Garcia-Montero AC, Sanchez L, et al. Recent advances in the understanding of mastocytosis: the role of KIT mutations. Br J Haematol. 2007;138(1):12-30. 3. Longley BJ, Reguera MJ, Ma Y. Classes of c-KIT activating mutations: proposed mechanisms of action and implications for disease classification and therapy. Leuk Res. 2001;25(7):571-6. 4. Garcia-Montero AC, Jara-Acevedo M, Teodosio C, et al. KIT mutation in mast cells and other bone marrow hematopoietic cell lineages in systemic mast cell disorders: a prospective study of the Spanish Network on Mastocytosis (REMA) in a series of 113 patients. Blood 2006; 108: 2366-72. 5. Kristensen T, Vestergaard H, Møller MB. Improved detection of the KIT D816V mutation in patients with systemic mastocytosis using a quantitative and highly sensitive real-time qPCR assay. J Mol Diagn. 2011;13(2):180-8. 6. Sperr WR, Valent P. Diagnosis, progression patterns and prognostication in mastocytosis. Expert Rev Hematol. 2012;5(3):261-74. 7. Roskoski R Jr. Structure and regulation of Kit protein-tyrosine kinase--the stem cell factor receptor. Biochem Biophys Res Commun. 2005 23;338(3):1307-15. 8. Gotlib J, DeAngelo SJ, George TI, et al. KIT Inhibitor Midostaurin Exhibits a High Rate of Clinically Meaningful and Durable Responses in Advanced Systemic Mastocytosis: Report of a Fully Accrued Phase II Trial. Blood. 2010;116(21):316. 9. da Silva EZ, Jamur MC, Oliver C. Mast cell function: a new vision of an old cell. J Histochem Cytochem. 2014;62(10):698-738.

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