HAEM4:Mastocytosis
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Primary Author(s)*
Shashi Shetty, Ph.D. FACMG, FCCMG, University Hospitals Cleveland Medical Center, Case Western Reserve University
Graphical Data Links
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General Disease Overview / Description of Cancer Category
Mastocytosis comprises a heterogeneous group of disorders characterized by expansion and accumulation of neoplastic mast cells (MCs) in one or more organ systems. In systemic mastocytosis (SM), neoplastic MCs form focal and/or diffuse infiltrates in various internal organs, including the bone marrow (BM), spleen, liver, and gastrointestinal tract. Regardless of the type of SM, the BM is involved in virtually all patients. Skin involvement is usually found in patients with indolent SM (ISM), is less frequently detected in aggressive SM (ASM), and is rarely seen in MC leukemia (MCL). Based on the classification of the World Health Organization (WHO), mastocytosis can be divided into cutaneous mastocytosis (CM), systemic mastocytosis (SM), and mast cell sarcoma.1,
WHO Classification Pages (Includes Links to Content)
Other Related Pages (Includes Links to Content)
http://www.ccga.io/index.php/HAEM5:Cutaneous_mastocytosis
Additional Information
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References
- Arber DA, et al., (2017). Mastocytosis, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p62-69.
Notes
*Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage). Additional global feedback or concerns are also welcome.