7. How is Mastocytosis Treated ?
There are three important ways of treating mastocytosis: i. prophylaxis and avoidance of triggering factors, ii. anti-mediator-drugs, and iii. cytoreductive therapies.
In many patients with mastocytosis, no symptoms occur - and thus no therapy may be required. Prophylactic anti-histamines has been proposed for such patients in some textbooks. In fact, prophylactic antihistamines may help to prevent histamine-related symptoms and disorders (like ulcerative stomach disease) even if no such symptoms have occurred before.
Another important aspect is the identification and avoidance of triggering factors = factors that may induce a severe anaphylactoid reaction. Because mastocytosis is a very heterogenous disease with divergent responses to triggering agents (i.e., what may trigger a reaction of mast cells in one person, may be harmless to someone else), it is very important for the patient to identify all potential triggers and to avoid them. Triggers can include allergens, insects, food, specific ingredients, environmental factors, friction, and also emotional stress. Once a patient is aware of the specific triggers that cause mast cells to degranulate, avoiding those triggers becomes part of the treatment plan.
Two important aspects are anaesthesia and allergy. In case of a known allergy, the (suspected) allergen must be strictly avoided. Before surgery, the doctors (i.e. both the surgeon and anaesthesiologist) should know that you are suffering from mastocytosis. The anesthesia should be adjusted to the presence of mastocytosis in each case. A preoperative intradermal skin test to predict the patients who may develop reactions is recommended.
A number of anti-mediator-type drugs are prescribed for patients with mastocytosis. The most commonly used drugs are anti-histamines i.e. histamine receptor antagonists. Two major groups of antihistamines are available, i.e. H1 and H2 receptor antagonists.
H1 antihistamines are drugs that are used to treat the symptoms of itching, hiving, and flushing. It can also bring some relief to gastrointestinal symptoms. Examples of H1 antihistamines are hydroxyzine, cetirizine, loratadine, or diphenhydramine.
H2 antihistamines are drugs that relieve gastrointestinal symptoms associated with mastocytosis. These are ulcer or reflux medications. Examples of H2 antihistamines include ranitidine and famotidine.
In addition to antihistamines, a number of other drugs are also used in patients with mastocytosis to ameliorate mediator-related symptoms. These drugs include glucocorticosteroids, so called mast cell stabilizers (like cromolyn sodium - may be used to treat diarrhea), or ketotifen, which is also a potent H1 antihistamine.
Another antimediator-type drug is epinephrine which helps during most severe anaphylactoid reactions with hypotension and shock. According to recommendations in several textbooks, all mastocytosis patients should carry emergency medicines with them including an epi-pen.
In each case, the treatment of mediator-related symptoms needs to be individualized by a physician who will do so according to the symptoms reported by patients. In general, the treatment is aimed at controlling symptoms with H1 and H2 anti-histamines alone (without other drugs).
Finally, it is important for the patient and for the doctors to have good communication and to work cooperatively on the goal to achieve the best possible control of disease. |
