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Future Oncol. 2018 Nov;14(26):2713-2723. doi: 10.2217/fon-2018-0291. Epub 2018 Sep 12.

The Italian Mastocytosis Registry: 6-year experience from a hospital-based registry.

Author information

1
Hematology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
2
Hematology Department, James Cook University Hospital, South Tees Trust (NHS), Middlesbrough, UK.
3
Dermatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
4
Hematology, Policlinico GB Rossi, Verona, Italy.
5
Dermatology, Department of Experimental, Diagnostic & Specialty Medicine, University of Bologna, Bologna, Italy.
6
Immunology & Allergology, University of Salerno, Salerno, Italy.
7
Pediatric Dermatology, DIMED, University of Padua, Padua, Italy.
8
Dermatololgy & Venerology, Hospital of Brunico, Italy.
9
Allergology & Immunology, Niguarda Hospital, Milano, Italy.
10
Center of Research & Innovation of Myeloproliferative Neoplasms (CRIMM), Careggi University Hospital/University of Florence, Florence, Italy.
11
Hematology, University of Bologna, Bologna, Italy.
12
Allergy Unit, Sant'Anna Hospital, Como, Italy.
13
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy.
14
Pediatric, University of Cagliari, Cagliari, Italy.
15
Hematology, San Raffaele Hospital, Milano, Italy.
16
Dermatology, University of Florence, Florence, Italy.
17
Hematology, University of Siena, Siena, Italy.
18
Dermatology, University of Parma, Parma, Italy.
19
Divisione di Ematologia, Ospedale San Gerardo, ASST Monza, Italy.
20
Hematology, Ospedale di Cesena, Cesena, Italy.
21
Gastroenterology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
22
Internal Medicine, IRCCS, Policlinico San Matteo, Pavia, Italy.
23
Pediatric Department, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
24
Pathology Department, Fondazione IRCCS Policlinico San Matteo, Pavia, University of Pavia, Italy.
25
Allergology, Ospedale Civile Maggiore, Verona, Italy.
26
Ematologia, Sapienza Università di Roma, Azienda Policlinico Umberto I Roma, Italy.
27
Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria.
28
Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.

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.

KEYWORDS:

hospital-based registry; mastocytosis; multidisciplinary management

PMID:
30207489
DOI:
10.2217/fon-2018-0291
[Indexed for MEDLINE]

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