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Future Oncol ; 14(26): 2713-2723, 2018 11.
Article in English | MEDLINE | ID: mdl-30207489

ABSTRACT

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.


Subject(s)
Mastocytosis, Cutaneous/epidemiology , Mastocytosis, Systemic/epidemiology , Registries/statistics & numerical data , Adolescent , Adult , Bone Marrow/pathology , Child , Female , Humans , Italy/epidemiology , Male , Mastocytosis, Cutaneous/diagnosis , Mastocytosis, Cutaneous/genetics , Mastocytosis, Cutaneous/pathology , Mastocytosis, Systemic/diagnosis , Mastocytosis, Systemic/genetics , Mastocytosis, Systemic/pathology , Mutation , Prevalence , Proto-Oncogene Proteins c-kit/genetics , Retrospective Studies , Skin/pathology , Tryptases/blood , Young Adult
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