Los síntomas de una mastocitosis sistémica (MS) afectan a la calidad de vida del paciente, incluso en las formas leves de la enfermedad.1-4
La sintomatología de la mastocitosis sistémica es muy heterogénea. Esta sintomatología puede ser debida a los síntomas provocados por la liberación de mediadores de mastocitos o a síntomas de organopatía, provocados por la infiltración de mastocitos, (o a una combinación de los dos), y observamos el siguiente espectro clínico:1-4
Los síntomas de una mastocitosis sistémica (MS) afectan a la calidad de vida del paciente, incluso en las formas leves de la enfermedad.1-4
Referencias:
1. 1. Pardanani A. Systemic mastocytosis in adults: 2019 update on diagnosis, risk stratification and management. Am J Hematol 2019; 94: 363-77. 2. Fuller SJ. New insights into the pathogenesis, diagnosis, and management of mastocytosis. Hematol Oncol Clin North Am. 2012;26(6):1143-68. 3. Valent P. Risk factors and management of severe life-threatening anaphylaxis in patients with clonal mast cell disorders. Clin Exp Allergy. 2014;44(7):914-20. 4. Horny H P, et al. Mastocytosis. In: Swerdlow S, et al, eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC; 2008:54 63. 5. Theoharides TC, Valent P, Akin C. Mast Cells, Mastocytosis, and Related Disorders. N Engl J Med. 2015;373(2):163-72. 6. Pardanani A. Systemic mastocytosis in adults: 2017 update on diagnosis, risk stratification and management. Am J Hematol. 2016;91(11):1146-59. 7. Arock M, Akin C, Hermine O, et al. Current treatment options in patients with mastocytosis: status in 2015 and future perspectives. European Journal of Haematology. 2015;94(6):474-90. 8. Haddad E, Joukhadar C, Chehata N, et al. Anaphylaxis as a manifestation of systemic mastocytosis: a case report and review of literature. J Clin Case Rep. 2017;7(2):1-4. 9. Jawhar M, Schwaab J, Horny HP, et al. Impact of centralized evaluation of bone marrow histology in systemic mastocytosis. Eur J Clin Invest. 2016;46(5):392-7. 10. Gülen T, Hägglund H, Dahlén B, et al. Mastocytosis: the puzzling clinical spectrum and challenging diagnostic aspects of an enigmatic disease. Journal of Internal Medicine. 2016;279(3):211-28. 11. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Systemic Mastocytosis V.2.2019. © National Comprehensive Cancer Network, Inc. 2018. All rights reserved. Accessed November 13, 2018. To view the most recent and complete version of the guideline, go online to NCCN.org.
