Local lesions may be excised.
Avoid triggers such as temperature extremes, physical exertion, or in some cases opiate analgesics, nonsteroidal antiinflammatory drugs, ingestion of ethanol containing drinks.
Anaphylaxis may follow stings by venomous insects. Epinephrine-filled syringes and instructions for self-administration should be carried by patients considered at risk. These patients may also benefit from prophylactic antihistamines in settings and during seasons that insect stings are prevalent. Patients with mastocytosis can also have anaphylaxis from iodinated contrast material.
Mastocytosis currently has no curative therapy and symptomatic therapy, although transiently helpful, does not alter course of the disease.
Histamine-2 (H2)-receptor antagonists (e.g., cimetidine, ranitidine, famotidine) can decrease gastric hyperacidity, and can be used to treat gastritis or peptic ulcer. Proton pump inhibitors may also be useful in treating gastric hypersecretion. They may, in combination with histamine-1 (H1)-receptor antagonists, contribute to ameliorating mast-cell constituent release–related signs and symptoms.
H1-receptor antagonists (e.g., diphenhydramine, chlorpheniramine, tricyclic antidepressants) can decrease flushing, vasodilation, and headache. More potent H1-receptor blockers (hydroxyzine and doxepin) may be useful in more severe cases.
Oral disodium cromoglycate may alleviate gastrointestinal cramping, diarrhea, and headache. It has also been useful in childhood cutaneous mast cell disease.
Ketotifen may ameliorate pruritus and wheal formation and may minimize osteoporosis.
Diphosphonates have also been used to stop or reverse osteopenia.
Cutaneous glucocorticoids and 8-methoxypsoralen and ultraviolet light (PUVA) have been reported to decrease pruritus or improve the appearance of skin lesions.
Oral glucocorticoids can be used for malabsorption or ascites. In adults, the doses used to start therapy are approximately 0.75 mg/kg for 2 to 3 weeks and then they are tapered, eventuating in alternate day use, if they are helpful.
Insufficient data are available to determine the usefulness of cytotoxic agents, such as cladrabine, for progressive mastocytosis. Chemotherapy, generally, has been disappointing in cases of aggressive systemic disease.
Allogeneic stem cell transplantation has been used very infrequently, especially if an associated clonal myeloid disease coexists, but the mastocytic component of disease seems not to have been benefitted, usually.
In all the therapy noted above, success is unpredictable, the treatment has its own consequential side effects, and one must exercise judgment continually about whether the treatment is resulting in a net benefit to the patient.
Avoid mast cell stimulants such as alcohol, anticholinergics, aspirin, other nonsteroidal antiinflammatory agents, and morphine derivatives.
Inhibitors of mutant KIT-encoded tyrosine kinase (e.g., imatinib mesylate) have been generally unsuccessful in therapy because the most prevalent KIT mutation at codon 816 (Asp816Val) is resistant to these drugs. Rare mutations such as KIT (Phe522Cys) are responsive. Some diseases not technically mastocytosis but with elevated mast cells and elevated serum tryptase and with the FIPIL1-PDGFRA fusion gene do respond. (e.g., chronic eosinophilic leukemia, see Chaps. 34 and 47). If possible, mutational analysis on the mastocytosis cells should be done to determine if a drug-sensitive mutated kinase is present.