medigraphic.com
ISSN 0301-696X (Print)
Órgano de difusión del Servicio de Sanidad Militar y del Colegio Nacional de Médicos Militares

2019, Number 2

<< Back Next >>

Rev Sanid Milit Mex 2019; 73 (2)

Aggressive systemic mastocytosis, one case report and review of the literature

Carrillo JÁ, García CC, Campos CÁ, Aguilar LJ, Cardoso YG, García CAS

Language: Spanish
References: 8
Page: 166-169
PDF size: 261.26 Kb.


ABSTRACT

Systemic mastocytosis is a disease which diagnosis cannot be made by clinical criteria alone, but based on morphological criteria. Clinical presentation can vary and be nonspecific because of the possible sites of involvement. We present de clinical case of a patient diagnosed with aggressive systemic mastocytosis in the Hospital Central Militar of Mexico City, treated with CHOP chemotherapy scheme. In smoldering cases, systemic mastocytosis can be treated with alpha interferon, but in aggressive ones, they usually need polichemotherapy and allogenic marrow transplantation to achieve a long term survival.


REFERENCES

  1. Horny HP, Akin C, Arber DA et al. Mastocytosis. In: Swerdlow SH, Campo E, Harris NL et al, eds. WHO Classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC Press; 2017. 62-69.

  2. Brockow K. Epidemiology, prognosis, and risk factores in mastocytosis. Immunol Allergy Clin North Am. 2014; 34: 283-295.

  3. Mahendraraj, Krishnaraj Gudipati, Saketh, Chamberlain, Ronald S. Malignant Mastocytosis: a rare and diverse disease- demographics and clinical outcomes for 349 patients from the surveillance, epidemiology and end results (SEER) database (1973-2010). Scientific poster presentations: 2014 Clinical Congress. J Am Coll Surg. 2014; 219 (4): 166.

  4. Horny HP. Systemic mastocytosis. In: Porwitt A. Blood and bone marrow pathology. 2011. pp. 381-90.

  5. Gotlib J, Horny HP, Valent P. Mast cells and mastocytosis. In: Hematology: basic principles and practice. 2017; 72: 1170-1186.

  6. Tremblay D, Carreau, N, Kremyanskaya M, Mascarenhans J. Systemic mastocytosis: clinical update and future directions. Clin Lymphoma Myeloma Leuk. 2015; 15 (12): 728-738.

  7. Ustun C, Arock M, Liun-Nelemans HC et al. Advanced systemic mastocytosis: from molecular and genetic progress to clinical practice. Haematologica. 2016; 101 (10): 1133-1143.

  8. Valent P, Sperr Wr, Akin C. How I treat patients with advanced systemic mastocytosis. Blood. 2010; 116 (26): 5812-5817.




2020     |     www.medigraphic.com