Neues zur therapie der mastozytose

Mastocytosis is characterized by an increase of mast cell numbers in the skin and/or in other tissues. Symptoms result mainly from the release of mast cell mediators, especially of histamine. A curative therapy is not available, however, treatment may eliminate or reduce symptoms of mild disease and may delay the progression of aggressive forms of disease. In the last two years, new therapeutic approaches have been developed on the area of anaphylaxis, topical therapy of cutaneous mastocytosis, and therapy of aggressive forms of mastocytosis with antineoplastic drugs and tyrosine kinase inhibitors. Anaphylaxis is not uncommon in adult patients with mastocytosis. Patients at risk for anaphylaxis need an emergency set for self-medication. For children, this is normally not required. General anaesthesia is normally well tolerated in children, when precautionary measures are taken. Specific Immunotherapy with insect venoms may be conducted in patients with mastocytosis. In cases of recurrent anaphylaxis, anti-IgE antibodies (Omalizumab) is a new option. New approaches for topical therapy of cutaneous mastocytosis have been studied. For aggressive forms of mastocytosis, new tyrosine kinase inhibitors and cytostatic drugs have been used with partial success. It may be expected that a combination of different cytostatic drugs will lead to better response rates. Recent insights into the pathogenesis and therapy of mastocytosis immensely broaden the therapeutic spectrum for mastocytosis, so that hopefully adequate treatment modalities will be available for this disease in future.