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Mastocytosis - a Layperson's Guide. Mast cells are present in us all and are essential in the fight against infection and invasion by foreign bodies. They are present in greater numbers in the areas which are most likely to encounter these infections or foreign invaders - the skin, gut and the lining of the respiratory system. Mastocytosis is caused by a proliferation of mast cells. These are made in the bone marrow, and one of the diagnostic tools is a biopsy of marrow cells. The cells produced are abnormal in shape, and not only are they produced in greater numbers in sufferers, but they are longer lived, giving rise to much larger numbers of mast cells than are present in other people. When these cells invade other organs the disease is described as Systemic. Mastocytosis is described as a heterogenous condition. This means that its cause is uncertain, and indeed may have a number of causative factors. It also means that the signs and symptoms of the disease vary greatly from sufferer to sufferer. It is important to know that the disease is not contagious, and is not transmitted from parent to offspring. Generally speaking, there are two types of mastocytosis, adult and paediatric. Paediatric mastocytosis (affecting children) usually affects the skin (cutaneous mastocytosis or urticaria) and very often disappears in adolescence or adulthood. Mast cells are involved in the body's immune response system, and contain a number of potent chemicals, perhaps the best known of which is Histamine. Histamine is contained in granules within the mast cell, and under certain circumstances the cells degranulate, releasing histamine and causing irritation and inflammation. If enough mast cells degranulate at the same time a condition called anaphylaxis can result. This is potentially very serious, and sufferers who are liable to anaphylactic attacks may carry emergency supplies of adrenaline with them in the form of an Epipen Another potentially very useful aid is the MEDICALERT bracelet. |