MAST CELL ACTIVATION DISORDER

MCAS_proposed_criteriaMast Cells are found in connective tissue.  Some disorders of idiopathic mast cell activation include anaphylaxis, chronic urticaria, intrinsic asthma and others.  Mast Cell Activation Disorders are not able to be clinically distinguished from Mastocytosis.  There are other factors known to be asociated with MCA, for example hypothyroidism, autoimmunity and neoplastic disease.

Systemic mastocytosis is a disorder caused by a genetic mutation that results in an excessive number of mast cells in the body. Mast cells normally help protect you from disease and help in wound healing by releasing substances such as histamine and leukotrienes. But with systemic mastocytosis, excess mast cells can build up in the skin, around blood vessels, in the respiratory, gastrointestinal and urinary tracts, or in reproductive organs. When triggered, these mast cells release substances that can overwhelm the body and result in symptoms such as facial flushing, itching, a rapid heartbeat, abdominal cramps, lightheadedness or even loss of consciousness. Common triggers include alcohol, temperature changes, spicy foods and certain medications.

The Mastocytosis Society Inc describes Mastocytosis as:

“Mastocytosis has been defined in the literature as an abnormal accumulation of tissue mast cells in one or more organ systems. Broadly separated into two categories – cutaneous mastocytosis (CM) and systemic mastocytosis (SM), the disease occurs in both children and adults. CM is a benign skin disease representing the majority of pediatric cases. It often resolves during puberty and is not associated with systemic involvement, but because only a small subgroup of people with CM display a KIT mutation, not much is known about the factors that contribute to the accumulation of mast cells in the skin. Alternatively, most adult patients are diagnosed with SM. Skin involvement, typically urticaria pigmentosa, is common in adult patients and can provide an important clue to accurate diagnosis. In all SM categories, the common histological marker is the clustering of mast cells in visceral organs.”

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Scholarly article: “Mast cell activation disease: a concise practical guide for diagnostic workup and therapeutic options.”  Gerhard J Molderings,corresponding author1 Stefan Brettner,2 Jürgen Homann,3 and Lawrence B AfrinJ Hematol Oncol. 2011; 4: 10.  Published online 2011 Mar 22. doi:  10.1186/1756-8722-4-10

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3069946/

 

FURTHER HELP:

Perhaps ask for serum Tryptase to be tested, to see if it is high…..

“Dysautonomia International Research Update: POTS, EDS, MCAS Genetics Research update on a gene associated with a POTS/dysautonomia, MCAS, EDS phenotype”, presented during Dysautonomia International’s 2015 Conference by Dr. Joshua Milner, Chief, Genetics and Pathogenesis of Allergy Section, Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Disease, National Institutes of Health. https://vimeo.com/142039306

 

Dr. Maitland’s 2014 EDNF Conference video: http://eds-nyc.com/tag/dr-maitland/

Dr Maitland’s slides about MCAS: http://ednf.org/sites/default/files/Anne%20Maitland.pdf

Dr. Mariana Castells: http://csfinfo.org/videos/physician-lecture-videos/greater-metropolitan-was hington-area-chapter-lectures/.

Pamphlet to learn more and to teach others about MCAS: http://mastocytosis.ca/MSC%20Information%20Pamphlet.pdf

Dr. Afrin, “A concise, practical guide to diagnostic assessment for mast cell activation disease”, World Journal of Hematology, 2014: http://www.wjgnet.com/2218-6204/pdf/v3/i1/1.pdf

Pictures of dermatographic urticaria:  http://en.m.wikipedia.org/wiki/Dermatographic_urticaria

See also: http://www.mastocytosis.ca/masto.htm

 

 

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