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

被引：9

作者：

Merante, Serena ^{[1
,2
]}

Ferretti, Virginia V. ^{[1
]}

Elena, Chiara ^{[1
]}

Brazzelli, Valeria ^{[3
]}

Zanotti, Roberta ^{[4
]}

Ner, Iria ^{[5
]}

Magliacane, Diomira ^{[6
]}

Belloni Fortina, Anna ^{[7
]}

Ingeborg, Forer ^{[8
]}

Pastorello, Elide A. ^{[9
]}

Pieri, Lisa ^{[10
]}

Papayannidis, Cristina ^{[11
]}

Mauro, Marina ^{[12
]}

Grifoni, Federica ^{[13
]}

Minelli, Roberto ^{[14
]}

Guggiari, Elena ^{[15
]}

Difonzo, Elisa ^{[16
]}

Bocchia, Monica ^{[17
]}

Caroppo, Francesca ^{[7
]}

Di Nuzzo, Sergio ^{[18
]}

Elli, Elena Maria ^{[19
]}

Rondoni, Michela ^{[20
]}

Ciccocioppo, Rachele ^{[21
]}

Di Stefano, Michele ^{[22
]}

Bossi, Grazia ^{[23
]}

Boveri, Emanuela ^{[24
]}

Bonadonna, Patrizia ^{[25
]}

Giona, Fiorina ^{[26
]}

Valent, Peter ^{[27
,28
]}

Triggiani, Massimo ^{[6
]}

机构：

[1] Fdn IRCCS Policlin San Matte, Hematol, Pavia, Italy

[2] James Cook Univ Hosp, South Tees Trust NHS, Hematol Dept, Middlesbrough, Cleveland, England

[3] Fdn IRCCS Policlin San Matteo, Dermatol, Pavia, Italy

[4] Policlin GB Rossi, Hematol, Verona, Italy

[5] Univ Bologna, Dept Expt Diagnost & Specialty Med, Dermatol, Bologna, Italy

[6] Univ Salerno, Immunol & Allergol, Salerno, Italy

[7] Univ Padua, Pediat Dermatol, DIMED, Padua, Italy

[8] Hosp Brunico, Dermatol & Venerol, Brunico, Italy

[9] Osped Niguarda Ca Granda, Allergol & Immunol, Milan, Italy

[10] Univ Florence, Careggi Univ Hosp, Ctr Res & Innovat Myeloproliferat Neoplasms CRIMM, Florence, Italy

[11] Univ Bologna, Hematol, Bologna, Italy

[12] St Anna Hosp, Allergy Unit, Como, Italy

[13] Fdn IRCCS Ca Grande Osped Maggiore Policlin Milan, Milan, Italy

[14] Univ Cagliari, Pediat, Cagliari, Italy

[15] Hosp San Raffaele, Hematol, Milan, Italy

[16] Univ Florence, Dermatol, Florence, Italy

[17] Univ Siena, Hematol, Siena, Italy

[18] Univ Parma, Dermatol, Parma, Italy

[19] ASST Monza, Osped San Gerardo, Div Ematol, Monza, Italy

[20] Osped Cesena, Hematol, Cesena, Italy

[21] Univ Pavia, Fdn IRCCS Policlin San Matteo, Gastroenterol, Pavia, Italy

[22] IRCCS, Policlin San Matteo, Internal Med, Pavia, Italy

[23] Fdn IRCCS Policlin San Matteo, Pediat Dept, Pavia, Italy

[24] Univ Pavia, Fdn IRCCS Policlin San Matteo, Pathol Dept, Pavia, Italy

[25] Osped Civile, Allergol, Verona, Italy

[26] Azienda Policlin Umberto I Roma, Sapienza Univ Roma, Ematol, Rome, Italy

[27] Med Univ Vienna, Div Hematol & Hemostaseol, Dept Internal Med 1, Vienna, Austria

[28] Med Univ Vienna, Ludwig Boltzmann Inst Hematol & Oncol, Vienna, Austria

来源：

FUTURE ONCOLOGY | 2018年 / 14卷 / 26期

关键词：

hospital-based registry; mastocytosis; multidisciplinary management; CELL ACTIVATION SYNDROMES; SYSTEMIC MASTOCYTOSIS; CONSENSUS PROPOSAL; CLASSIFICATION; MANAGEMENT; DIAGNOSIS; INDOLENT; CRITERIA; MUTATION; BLOOD;

D O I：

10.2217/fon-2018-0291

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

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 >20ng/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.

引用

页码：2713 / 2724

页数：12

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