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, S., Ferretti, V.V., Elena, C., Brazzelli, V., Zanotti, R., Ner, I., Magliacane, D., Belloni Fortina, A., Ingeborg, F., Pastorello, E.A., Pieri, L., Papayannidis, C., Mauro, M., Grifoni, F., Minelli, R., Guggiari, E., Difonzo, E., Bocchia, M., Caroppo, F., Di Nuzzo, S., et al.. - 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.
