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Mastocytosis FAQ
Mastocytosis
Mastocytosis is a disorder that may 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.
Mastocytosis is a disorder that may 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. Mast cells seem to have other roles as well. Found to
gather around wounds, they may play a part in wound healing. For
example, the typical itching you feel 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. No one with too
few or no mast cells has ever been found. This fact indicates to some
scientists that having too few mast cells may be incompatible with life. The presence of too many mast cells, or mastocytosis, can
occur in two forms—cutaneous and systemic. The most common cutaneous
(skin) form is also called urticaria pigmentosa, which occurs when mast
cells infiltrate the skin. Systemic mastocytosis is caused by mast
cells accumulating in the tissues and can affect organs such as the
liver, spleen, bone marrow, and small intestine. Researchers first described urticaria pigmentosa in 1869.
Systemic mastocytosis was first reported in the scientific literature
in 1949. The true number of cases of either type of mastocytosis
remains unknown, but mastocytosis generally is considered to be an
“orphan disease.” (Orphan diseases affect approximately 200,000 or
fewer people in the United States.)
Chemicals released by mast cells cause changes in your
body’s functioning that lead to typical allergic responses such as
flushing, itching, abdominal cramping, and even shock. When too many
mast cells are in your body, the additional chemicals can cause It can also cause episodes of hypotension (very low blood pressure and faintness) or anaphylaxis (shock).
Your doctor can diagnose cutaneous mastocytosis by
the appearance of your skin and confirm it by finding an abnormally
high number of mast cells on a skin biopsy. The diagnosis of systemic mastocytosis is made when an increased number of abnormal mast cells is found during an examination of your bone marrow. Other tests that are important in evaluating a suspected
case of mastocytosis include measurement of a protein (tryptase) from
mast cells in your blood and a search for specific genetic mutations
that health experts associate with this disease.
Doctors use several medicines to treat mastocytosis
symptoms, including antihistamines (to prevent the effect of mast cell
histamine) and anticholinergics (to relieve intestinal cramping). A
number of medicines treat specific symptoms of mastocytosis. In cases in which mastocytosis is malignant, cancerous, or
associated with a blood disorder, steroids and/or chemotherapy may be
necessary. *cited from: www.niaid.nih.govOverview
Symptoms
Diagnosis
Treatment
