Aim: We collected 'real-life' data on the management of patients with mastocytosis in the Italian Mastocytosis Registry.
Methods: Six hundred patients diagnosed with mastocytosis between 1974 and 2014 were included from 19 centers.
Results: Among adults (n = 401); 156 (38.9%) patients were diagnosed with systemic mastocytosis. In 212 adults, no bone marrow studies were performed resulting in a provisional diagnosis of mastocytosis of the skin. This diagnosis was most frequently established in nonhematologic centers. In total, 182/184 pediatric patients had cutaneous mastocytosis. We confirmed that in the most patients with systemic mastocytosis, serum tryptase levels were >20 ng/ml and KIT D816V was detectable.
Conclusion: The Italian Mastocytosis Registry revealed some center-specific approaches for diagnosis and therapy. Epidemiological evidence on this condition is provided.
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| http://dx.doi.org/10.2217/fon-2018-0291 | DOI Listing |
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