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: 6-year experience from a hospital-based registry / Merante, Serena; Ferretti, Virginia V; Elena, Chiara; Brazzelli, Valeria; Zanotti, Roberta; Ner, Iria; Magliacane, Diomira; Belloni Fortina, Anna; Ingeborg, Forer; Pastorello, Elide A; Pieri, Lisa; Papayannidis, Cristina; Mauro, Marina; Grifoni, Federica; Minelli, Roberto; Guggiari, Elena; Difonzo, Elisa; Bocchia, Monica; Caroppo, Francesca; Di Nuzzo, Sergio; Elli, Elena Maria; Rondoni, Michela; Ciccocioppo, Rachele; Di Stefano, Michele; Bossi, Grazia; Boveri, Emanuela; Bonadonna, Patrizia; Giona, Fiorina; Valent, Peter; Triggiani, Massimo. - In: FUTURE ONCOLOGY. - ISSN 1479-6694. - 14:26(2018), pp. 2713-2723. [10.2217/fon-2018-0291]
The Italian Mastocytosis Registry: 6-year experience from a hospital-based registry
Di Nuzzo, SergioMembro del Collaboration Group
;
2018-01-01
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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
