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What is Mastocytosis?

Mastocytosis is a rare disease with an unknown cause, that affects both children and adults. It is a result of having too many mast cells in the body. We all have mast cells throughout our body. They are found in places like the liver, the bone marrow, the intestines, the spleen, and the skin. They play an important role in wound healing. They also help defend these tissues from disease by releasing chemical "alarms", like histamine. In mastocytosis, the body has too many mast cells, and they release more of these chemical alarms than the body requires. The symptoms of mastocytosis are caused by the excessive amounts of chemicals interacting with body tissue.

What are the symptoms of Mastocytosis?

When mast cells are triggered, they can degranulate; degranulating causes them to release their contents (their chemicals). They can release their contents all at once or they can leak the contents slowly in response to a trigger. It is important to treat symptoms as soon as they occur with Benadryl, as they can gradually get worse and become life threatening if left untreated. In the event of a sudden release of all contents, an epinephrine injection (Epi- Pens) would be required. Epi-pens are also required if Benadryl does not sufficiently control the reaction. The following are common symptoms of a mast cell reaction:

• Abdominal pain
• Nausea
• Cramping
• Diarrhea
• GERD
• Vomiting
• Malabsorbtion
• Inflammation of esophagus, stomach, and/or intestines
• Hives and other rashes
• Itching with and without rashes
• Flushing (turning red)
• Anaphalactic Shock and Cardiovascular Collapse
• Blood pressure changes
• Rapid heart rate
• Bone and/or muscle pain (mild to severe/debilitating)
• Dizziness/vertigo/lightheadedness, faintness
• Fatigue
• Peripheral neuropathy and paresthesias
• Hematological (blood) abnormalities

What are common triggers?

• Heat
• Cold
• Extreme or sudden temperature change
• Severe emotional distress
• Prescription medications like Opiates (codeine, vicodin, morphine, verced, and Demerol);
• Over the counter medications like Motrin, Neosporin, aspirin, cough medicine, and decongestants
• Artificial preservatives, dyes, and flavors in foods and medications
• Viruses and bacterial infections
• Immunizations
• Antibiotics
• Anything to which the individual has a known allergy 

What are the different forms of Mastocytosis?

Mastocytosis can take several forms, varying from mild forms to more severe forms of the disease. Any form of the disease presents challenges to raising children, and educating others on how mastocytosis affects their lives. The disease is divided into several categories.

Cutaneous (Skin):

(1) Urticaria Pigmentosa (UP): This is the most common presentation of Matocytosis. It involves small brown "spots" on the skin, and they can be found anywhere on the body. These spots flush and hive when rubbed or irritated by a trigger.

2) Solitary Mastocytoma: This is an accumulation of mast cells that resembles a tumor. It is one larger spot, rather than in UP, where there are many smaller ones. This also turns red and hives when irritated.

(3) Diffuse Cutaneous: An infiltration of mast cells covering the entire skin. This is the most rare form of the disease.

Systemic:

(1) Indolent Mastocytosis: The most common form of systemic. It is in the system, and effects your whole body, but is not said to shorten your life span.

(2) Mastocytosis with an associated Hematological (blood) Disorder

(3) Aggressive Mastocytosis: Significantly shortens your life span.

(4) Mast Cell Leukemia: Very aggressive form of leukemia with a short life span and no cure.

How many people have Mastocytosis?

The number of patients with Mastocytosis is unknown, because so few doctors recognize it.

What is the treatment and prognosis? Is there a cure?

Prognosis varies greatly depending on the form of the disease. Most children with the skin form of the disease will outgrow it by adolesence. It is very rare for a child to have the systemic form, but in the event that this occurs, he or she have it for his or her entire life.

There is no cure. Treatment is limited to avoidance of triggers and managing symptoms with medications. Examples of medications that are used to treat symptoms are Zantac (blocks the effects of histamine in the stomach), antihistamines (like Zyrtec, Periactin, Atarax, and Extendryl), leukotrine inhibitors (like Singulair), and Gastrocrom (a mast cell stabilizer). In the event that these medications are unable to control the symptoms, Benadryl and Epinephrine injections (Epi-Pens) are used as rescue medications to prevent death secondary to anaphylactic shock or a cardiovascular collapse. ALL MASTOCYTOSIS PATIENTS, REGARDLESS OF TYPE, SHOULD CARRY AN EPI PEN AT ALL TIMES.

Links

For additional information, the following webites are helpful:

www.tmsforacure.org and www.mastokids.org

Written by: Carla, MWP Member

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