Mast cell tumor medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Medical Therapy
There is currently no cure for mastocytosis. The treatment is palliative and there are a number of medicines to help treat the symptoms of mastocytosis:
Antihistamines
- Antihistamines block receptors targeted by histamine released from mast cells. Both H1 and H2 blockers may be helpful.
- Leukotriene antagonists block receptors targeted by leukotrienes released from mast cells.
Mast cell stabilizers
- Mast cell stabilizers help prevent mast cells from releasing their chemical contents. Cromolyn Sodium Oral Solution (Gastrocrom® / Cromoglicate) is the only medicine specifically approved by the U.S. FDA for the treatment of mastocytosis. Ketotifen is available in Canada and Europe, but is only available in the U.S. as ophthamic drops (Zaditor®).
Proton pump inhibitors
- Proton pump inhibitors help reduce production of gastric acid, which is often increased in patients with mastocytosis. Excess gastric acid can harm the stomach, esophagus, and small intestine.
Epinephrine
- Epinephrine constricts blood vessels and opens airways to maintain adequate circulation and ventilation when excessive mast cell degranulation has caused anaphylaxis.
Albuterol
- Albuterol and other beta-2 agonists open airways that can constrict in the presence of histamine.
Steroid
- Corticosteroids can be used topically, inhaled, or systemically to reduce inflammation associated with mastocytosis.
Antidepressants
- Antidepressants are an important and often overlooked tool in the treatment of mastocytosis. The stress and physical discomfort of any chronic disease may increase the likelihood of a patient developing depression. Depression and other neurological symptoms have been noted in mastocytosis.[1] Some antidepressants such as doxepin are themselves potent antihistamines and can help relieve physical as well as cognitive symptoms.
Calcium channel blocker
- Dihydropyridines are calcium channel blockers that are sometimes used to treat high blood pressure. At least one clinical study suggested that Nifedipine, one of the dihydropyridines, may reduce mast cell degranulation in patients that exhibit urticaria pigmentosa. A 1984 study by Fairly et al. included a patient with symptomatic urticaria pigmentosa who responded to nifedipine at dose of 10 mg po tid.[2] However, Nifetipine has never been approved by the FDA for treatment of mastocytosis.
The treatment of systemic mastocytosis is mainly focused on avoidance of triggering factors (e.g. physical stimuli such as heat or cold, alcohol, drugs such as aspirin and other NSAIDS) and symptomatic therapy (H1 and H2 antihistamines, proton pump inhibitors, antileukotrienes, anticholinergics, glucocorticoïds, and epinephrine in case of systemic hypotension). In aggressive forms of systemic mastocytosis, treatments such as interferon alpha, cladribin, and imatinib mesylate should to be considered. Imatinib seems to be more effective in patients without the D816V C-kit mutation.[3]
Healthcare providers use several medicines to treat mastocytosis symptoms, including antihistamines (to prevent the effect of mast cell histamine—a chemical) and anticholinergics (to relieve intestinal cramping). A number of medicines treat specific symptoms of mastocytosis.
- Antihistamines frequently treat itching and other skin complaints.
- Certain antihistamines work specifically against ulcers; proton pump inhibitors also relieve ulcer-like symptoms.
- Epinephrine treats symptom flares which occur with shock, referred to as "anaphylaxis."
- Two types of antihistamines treat severe flushing and low blood pressure before symptoms appear.
- Steroids treat malabsorption, or impaired ability to take in nutrients.
- Cromolyn sodium may help reduce cramping in the abdomen.
In cases in which mastocytosis is malignant, cancerous, or associated with a blood disorder, steroids and/or chemotherapy may be necessary.
References
- ↑ Rogers MP, Bloomingdale K, Murawski BJ, Soter NA, Reich P, Austen KF. Mixed organic brain syndrome as a manifestation of systemic mastocytosis. Psychosom Med 1986;48:437-47. PMID 3749421
- ↑ Fairley JA, et al: Urticaria pigmentosa responsive to nifedipine. J Am Acad Dermatol 11:740-743, 1984.
- ↑ Koenig, Martial; Morel, Jérôme; Reynaud, Jacqueline; Varvat, Cécile; Cathébras, Pascal (2008). "An unusual cause of spontaneous bleeding in the intensive care unit – mastocytosis: a case report". Cases Journal. 1 (1): 100. doi:10.1186/1757-1626-1-100. ISSN 1757-1626.