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, 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 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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