Mastocytosis is a group of rare disorders of both children and adults caused by the presence of too many mast cells (mastocytes) and CD34+ mast cell precursors in a person's body.
Mast cells are located in connective tissue, including the skin, the linings of the stomach and intestine, and other sites. They play an important role in helping defend these tissues from disease. By releasing chemical "alarms" such as histamine, mast cells attract other key players of the immune defense system to areas of the body where they are needed.
Mast cells seem to have other roles as well. Because they gather together around wounds, mast cells may play a part in wound healing. For example, the typical itching felt around a healing scab may be caused by histamine released by mast cells. Researchers also think mast cells may have a role in the growth of blood vessels (angiogenesis). No one with too few or no mast cells has been found, which indicates to some scientists that we may not be able to survive with too few mast cells.
Mast cells express a cell surface receptor termed c-kit (CD117), which is the receptor for scf (stem cell factor). In laboratory studies, scf appears to be important for the proliferation of mast cells, and inhibiting the tyrosine kinase receptor with imatinib (see below) may reduce the symptoms of mastocytosis.
Mast cell disease is divided into two broad categories, cutaneous and systemic:
Scientists first described urticaria pigmentosa in 1869. Systemic mastocytosis was first reported by scientists in 1936.
In some rare cases chemicals released by mast cells cause changes in the immune system leading to typical allergy symptoms such as:
Anaphylaxis (shock from allergic or immune causes)
When too many mast cells exist in a person's body, the additional chemicals can cause:
Episodes of very low blood pressure (including shock) and faintness
Bone or muscle pain
Nausea and vomiting
I have the cutaneous type called Urticaria Pigmentosa which just basically means I have more mast cells than most people do and they group together and look like reddish brown spots on my body. If they are touched then they become inflamed and send out histamine which causes me to itch. After about ten minutes it calms back down and I'm fine. I've had it all my life and mine isn't severe so it doesn't bother me. Other people have more severe cases and can be disabled by it (I believe) especially if they have the systemic type. I hope this helps you! Have a great day!
Mastocytosis is a disorder that can occur in both children and adults. It is caused by the presence of too many mast cells in your body. You can find mast cells in skin, lymph nodes, internal organs (such as the liver and spleen) and the linings of the lung, stomach, and intestine. Mast cells play an important role in helping your immune system defend these tissues from disease.
Mast cells attract other key players of the immune defense system to areas of your body where they are needed by releasing chemical “alarms” such as histamine and cytokines.
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