Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Mastocytosis

  • Srdan Verstovsek
  • Cem Akin
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_3549-2

Definition

Mastocytosis, a hematopoietic neoplasm, is a clonal disorder of the mast cell and its progenitor resulting in the presence of excessive numbers of mast in the skin, bone marrow, and internal organs such as the liver, spleen, and lymph nodes.

Characteristics

Most patients with mastocytosis present with symptoms due to the release of mediators from activated mast cells in various tissues. The activation of mast cells can be caused by triggers such as temperature changes, emotional or physical stress, exercise, ingestion of alcohol, spicy foods, or mast cell degranulating drugs, insect venom, and allergens, but may also occur without an apparent stimulus. Patients with mastocytosis display a wide range of clinical presentations, ranging from no symptoms to severe life-threatening syncopal episodes or hematologic abnormalities. The most common symptoms of mast cell degranulation are itching, flushing, gastrointestinal symptoms such as heartburn and diarrhea, lightheadedness, and...

Keywords

Mast Cell Systemic Mastocytosis Tryptase Level Urticaria Pigmentosa Mast Cell Leukemia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

  1. Butterfield JH (2006) Systemic mastocytosis: clinical manifestations and differential diagnosis. Immunol Allergy Clin North Am 26:487–513. http://dx.doi.org/10.1016/j.iac.2006.05.006
  2. Escribano L, Akin C, Castells M et al (2006) Current options in the treatment of mast cell mediator-related symptoms in mastocytosis. Inflamm Allergy Drug Targets 5:61–77. http://dx.doi.org/10.2174/187152806775269303
  3. Pardanani A, Akin C, Valent P (2006) Pathogenesis, clinical features, and treatment advances in mastocytosis. Best Pract Res Clin Haematol 19:595–615. http://dx.doi.org/10.1016/j.beha.2005.07.010
  4. Quintas-Cardama A, Aribi A, Cortes J et al (2006) Novel approaches in the treatment of systemic mastocytosis. Cancer 107:1429–1439. http://dx.doi.org/10.1002/cncr.22187
  5. Robyn J, Metcalfe DD (2006) Systemic mastocytosis. Adv Immunol 89:169–243. http://dx.doi.org/10.1016/s0065-2776(05)89005-4

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Leukemia DepartmentUniversity of Texas MD Anderson Cancer CenterHoustonUSA
  2. 2.University of MichiganAnn ArborUSA