What is Mastocytosis

Mast Cells
Mast cells are located normally in nearly all tissues in the body, and their greatest concentration is in the gastro-intestinal tract (GI tract), the skin, and the lining of the respiratory system.

Mast cells are involved in protection against infection, tissue repair and growth, control of blood flow into areas where it is needed, and communication with other cells of the immune system. Mast cells contain little packets of chemicals that can be released immediately when required. When they are released, the cell is said to “degranulate”.

Mastocytosis is the abnormal accumulation of mast cells (more than normal) that degranulate abnormally in response to multiple triggers.

Mast Cell Activation Disorder (MCAD)
Not a lot is known about this newly identified mast cell disease, but criteria will be formed sometime in 2011 by mast cell disease researchers. We know that people with MCAD have a normal or near normal amount of mast cells in their body, but these cells degranulate abnormally in response to multiple triggers. 

The symptoms of MCAD and Mastocytosis clinically present in an identical matter, and distinguishing between the two conditions can only be done through biopsies of the skin and/or bone marrow. 

Symptoms Mast cell degranulation can be triggered by temperature changes, heat, cold, humidity, exercise, sunlight, emotional stress, infections, and certain drugs and foods. Sometimes the trigger is unknown. When degranulation occurs, it is known as “a reaction”. Mild reactions typically cause only a few symptoms that feel flu-like and more severe reactions cause anaphylaxis, and even shock. Common symptoms include:

*Behavior changes (extreme irritability, inconsolable crying, and lack of reasoning ability)
*Flushing (facial redness)
*Facial swelling
*Hives and other rashes
*Itching
*Abdominal pain
*Bone and muscle pain
*Headache
*Fatigue
*Diarrhea
*Vomiting, retching, or severe acid reflux
*Nausea
*Difficulty breathing
*Low blood pressure (causes dizziness, palor, lethargy, weakness)
*Shock, or cardiovascular collapse (heart stops pumping blood resulting in death)

A serious complication of mastocytosis is prolonged ulcerative bleeding in the GI tract secondary to high levels of circulating plasma histamine. Almost all the symptoms of mastocytosis are caused by an increase of mast cell chemicals, or mediators, in the system.

TreatmentAvoidance of triggers and daily medications are used to prevent the effects of the circulating chemicals:

*Histamine 1 (H1) blockers-- like Atarax, Zyrtec, and Periactin
*Histamine 2 (H2) blockers-- like Zantac and Pepcid
*Leukotrine Inhibitors-- like Singulair and Zyflo
*Mast cell stabilizers-- like Gastrocrom and Ketotifen
*Proton pump inhibitors-- like Prevacid, Nexium, and Prilosec

Additional medications are given to treat an acute reaction:
*(H1) blocker (Benadryl)
*(H2) blocker (Zantac)
*Mast cell stabilizer (Gastrocrom)
*Inhaled bronchodilator (Albuterol)
*Epinephrine injections (Epi pen Jr.)

For more information on Mastocytosis, click on any of the following links:
The Mastocytosis Society~ www.tmsforacure.org
The National Institute of Health~ http://www.niaid.nih.gov/topics/mastocytosis/Pages/Default.aspx