Mastocytosis is defined as a myeloproliferative neoplastic (mpn) stem cell disorder, caused by an over-abundance of good immune
system cells called mast cells and the release of mast cell mediators. These cells may also be misshapen or malformed, further contributing
to their dysfunction.1 Mastocytosis presents in cutaneous (skin) and systemic (internal) forms.
Patients of all ages and genders may present with one or both forms of the disease.
The World Health Organization (WHO) has classified mastocytosis as having 7 variants with 13 subvariants2 & 3. See table below
(Note: we have linked to photographic images of our members photos of cutaneous forms):-|
|Mast Cell Activation Syndrome (MCAS) is constantly under discussion
with ongoing research in the medical community worldwide. The current definition
is that MCAS is caused by abnormal mast cells4 and/or abnormal mast cell activity5. We recommend viewing
the Mast Cell Activation medical lecture we hosted in June 2011 at University of Toronto, Ontario, Canada. See our Physicians & Emergency
Literature page to view here.
Idiopathic Anaphylaxis (IA) is defined as anaphylaxis which occurs without an identifiable trigger6. Photographic images
of our members in the throes of anaphylaxis may be viewed here.
- Arock,M - Laboratory of Cellular and Molecular Hematology, Faculty of Pharmacy, Paris: Mastocytosis: classification, laboratory diagnosis and treatment
Annals de Biologie Clinique, Epileptic Disorders. Volume 62, Number 6, 657-69 November-December 2004. [Open Access - French]
- McNeill,O., Katelaris,C.: Disease Summaries - Mastocytosis: Where are we now? World Allergy Organization July 2011.
- Nguyen,N.: Pathology: Mastocytosis (Mast Cell Diseases). The University of Texas, Health Science Center at
Houston 2012. [Open Access]
- Molderings,G., Brettner,S., Homann,J., Afrin,L.: Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options.
J Hematology & Oncology March 2011. [Open Access]
- Valent.P., Horny,H-P., Triggiani.M., Arock,M.: Clinical and Laboratory Parameters of Mast Cell Activation as Basis for the
Formulation of Diagnostic Criteria Int Arch Allergy Immunol 2011;156:119-127. May 2011. [Open Access]
- Greenberger,P.A: Idiopathic anaphylaxis Immunol Allergy Clin North Am.;27(2):273-93, vii-viii. May 2007. [Abstract]