[Systemic mastocytosis--new therapeutic strategies].
Systemic mastocytosis is characterized by an excessive proliferation of mast cells and their accumulation in different organs. Avoidance of trigger factors leading to anaphylaxis is a general measure valid for all forms of mastocytosis. A premedication is necessary in case of surgery, anesthesia or administration of radiocontrast agents. Symptomatic treatment comprises antihistamines, anti-leukotrienes, proton pump inhibitors and topical corticosteroids. Indolent mastocytosis with refractory symptoms, the rare cases of aggressive mastocytosis with organ dysfunction and the even rarer mast cell leukemia require cytoreductive therapy. First-line agents are interferon alpha 2b and imatinib, a tyrosine kinase inhibitor. To date there is no curative treatment.
Service d'immunologie et allergie, CHUV, 1011 Lausanne. christa-maria.maniu@chuv.ch
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D000486:Allergy and Immunology / Q000639:trends*; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D017338:Cladribine / Q000627:therapeutic use; D006801:Humans; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use; D034721:Mastocytosis, Systemic / Q000175:diagnosis / Q000517:prevention & control / Q000628:therapy*; D008495:Medical Oncology / Q000379:methods / Q000639:trends; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D011994:Recombinant Proteins / Q000627:therapeutic use; D035703:Therapies, Investigational / Q000379:methods* / Q000639:trends
D000893:Anti-Inflammatory Agents; D000077190:Interferon alpha-2; D016898:Interferon-alpha; D047428:Protein Kinase Inhibitors; D011994:Recombinant Proteins; D017338:Cladribine