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Mast Cell Disease
Treatment
Cutaneous lesions involving a limited area of the body may be treated with a topical corticosteroid, with occlusion if required. Intralesional injection of small amounts of dilute corticosteroids may help resolve skin lesions. The utility of systemic corticosteroids is limited to patients with manifestations such as ascites, malabsorption, and severe skin disease.1 Severe, unresponsive cases of cutaneous mastocytosis may require judicious use of oral psoralen plus UV-A (PUVA) therapy.1
Systemic mastocytosis can be initially addressed with antihistamines and corticosteroids to treat symptoms related to the release of mast cell mediators, to prevent or ameliorate anaphylaxis.2 Anticholinergic agents, aspirin, and other prostaglandin D2 inhibitors as well as photochemotherapy are also used in the treatment of systemic mastocytosis. Epinephrine can treat acute episodes of hypotension. Severe cases of systemic mastocytosis require treatment of the underlying hematologic disorder with chemotherapy or bone marrow transplantation. Splenectomy can be performed; however, this measure improves survival rates only slightly.3 IFN-alpha induces long-term responses in some patients with systemic mastocytosis, but has yet to be shown to be associated with prolonged survival.4
Course
Most patients with mastocytosis have an excellent prognosis.1 However, the disease course of systemic mastocytosis can range from presentation of only minor symptoms to progression into a life-threatening condition.5
The degree of tissue infiltration by mast cells has been correlated with disease aggressiveness. Malignant cell infiltration into organs may cause cytopenias, osteoporosis, pathologic fractures, hepatosplenomegaly, lymphadenopathy, and malabsorption.6 Median survival of patients with malignant forms of systemic mastocytosis is 1 to 2 years following diagnosis.5
Emerging Therapies
Recent advances in the understanding of the molecular pathogenesis of systemic mast cell disease have led to the development of a new therapeutic approach based on targeted inhibition of tyrosine kinase. This approach shows considerable promise in the management of patients with this disorder. Clinical trials are underway.
Clinical Trials
For U.S. Residents Only
References:
- Hogan D. Mastocytosis. Available at: http://www.emedicine.com/derm/topic258.htm. Accessed April 23, 2006.
- Hartmann K, Henz BM. Mastocytosis: recent advances in defining the disease. Br J Dermatol. 2001;144:682-695.
- Worobec AS, Metcalfe DD. Mastocytosis: current treatment concepts. Int Arch Allergy Immunol. 2002;127:153-155.
- Tefferi A, Pardanani A. Imatinib therapy in clonal eosinophilic disorders, including systemic mastocytosis. Int J Hematol. 2004;79:441-447.
- Cortes J, Kantarjian H. Beyond chronic myelogenous leukemia: potential role for imatinib in Philadelphia-negative myeloproliferative disorders. Cancer. 2004;100:2064-2078.
- Hennessy B, Giles F, Cortes J, et al. Management of patients with systemic mastocytosis: review of M.D. Anderson Cancer Center experience. Am J Hematol. 2004;77:209-214.
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