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.
The Italian Mastocytosis Registry: a 6-year experience of a hospital-based registry / Merante, S; Ferretti, Vv; Elena, C; Brazzelli, V; Zanotti, R; Ner, I; Magliacane, D; Belloni Fortina, A; Ingeborg, F; Pastorello, Ea; Pieri, L; Papayannidis, C; Mauro, M; Grifoni, F; Minelli, R; Guggiari, E; Difonzo, E; Bocchia, M; Caroppo, F; Di Nuzzo, S; Elli, Em; Rondoni, M; Ciccocioppo, R; Di Stefano, M; Bossi, G; Boveri, E; Bonadonna, P; Giona, F; Valent, P; Triggiani, M. - In: FUTURE ONCOLOGY. - ISSN 1082-331X. - 14:26(2018), pp. 2713-2723. [10.2217/fon-2018-0291]
The Italian Mastocytosis Registry: a 6-year experience of a hospital-based registry.
Giona F;
2018
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.| File | Dimensione | Formato | |
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