Mastocytosis is a kind of rare disorder which occurs due to presence of large number of mast cells and CD34 mast cells. It affects children and adults equally. Also, men and women are affected in same ratio. Mastocytosis is often referred as “ Orphan Disease” .
Connective tissues of skin, lining of stomach, intestine and other sites, contain mast cells. Mast cells are important for protecting these tissues from any disease. Mast cells release chemical like histamines which act like alarm to attract other vital elements of immune defense system to the area affected.
Mast cells are important for healing of a wound; they surround the wound. Histamines released by mast cells create that itching feeling around eth healing wound. It is likely that mast cells also play a role in growth of blood vessels.
Mastocytosis or the presence of too many mast cells can have different forms.
Cutaneous Mastocytosis is the one in which mast cells are present in skin only. The most common form of cutaneous mastocytosis is urticaria pigmentosa which mostly affects children. The rare form of cutaneous mastocytosis, which affects adults, is Telangiectasia Macularis Eruptiva Perstans (TMEP).
Other type of mastocytosis is systematic mastocytosis. This affects internal organs of the body like liver, lymph nodes, bone marrow and spleen including skin.
Immune system gets some changes by release of chemicals. This leads to allergy symptoms which includes itching, abdominal cramping and anaphylaxis.
Additional chemicals release by presence of too many mast cells in a person’s body can cause following symptoms: Skin Lesions, Abdominal Discomfort, faintness, low blood pressure, nausea, vomiting and muscle and bone pain.
Mastocytosis can affect people of any age group. There are namany natural chemicals in mastocytosis like histamines. There are many factors which can cause release of these chemicals. These factors include rubbing, heat, certain foods and certain medicines. Mastocytosis can be mild to severe in its form.
Family history of mastocytosis
Presence of excess of mast cells in the body
Any abnormality in the blood
Instances of having very low blood pressure
C-kit receptor mutation
Urticaria pigmentosa is identified by presence of fixed dark brown lesions. To confirm it, a skin biopsy can be recommended.
Systematic mastocytosis can be diagnosed by taking a biopsy from different organ like bone marrow.
Doctor can also go for a blood test and urine test which can indicate high level of certain mast-cell chemicals and proteins in person’s blood.
Mastocytosis can not be cured but there are many treatments which can reduce the symptoms related to mastocytosis. In case of mastocytosis in children, a mastocytoma disappears spontaneously. The most important part of treatment is its prevention; try to stay away from stimulants which trigger your symptoms of mastocytopsis.
To control the itching caused in urticaria pigmentosa, antihistamines are advised.
Corticosteroids can be advised to reduce the inflammation caused by mastocytosis. Corticosteroids can be applied topically, can be inhaled or can be taken orally.
Mastocytosis causes excessive secretion of gastric acid and this production of gastric acid can be reduced by proton pump inhibitors. Stomach, small intestine and esophagus get damaged by excessive gastric acid.
Excessive degranulation of too many mast cells causes anaphylaxis which obstructs airway. To have adequate circulation and ventilation, epinephrine is given to open the airway and constricts blood cells.
Airway can constrict in presence of histamines and Albuterol is given to open the airway.
Signs of depression are often seen in people suffering with mastocytosis. This is because of eth stress and physical discomfort created by this kind of chronic disease. Antidepressants are administered to reduce these symptoms. Some of these antidepressants are strong antihistamines also and can relieve physical and cognitive symptoms of mastocytosis.
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