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

共 26 条

[1] Nonaggressive systemic mastocytosis (SM) without skin lesions associated with insect-induced anaphylaxis shows unique features versus other indolent SM [J].

Alvarez-Twose, Ivan ;

Zanotti, Roberta ;

Gonzalez-de-Olano, David ;

Bonadonna, Patrizia ;

Vega, Arantza ;

Matito, Almudena ;

Sanchez-Munoz, Laura ;

Mario Morgado, Jose ;

Perbellini, Omar ;

Garcia-Montero, Andres ;

De Matteis, Giovanna ;

Teodosio, Cristina ;

Rossini, Maurizio ;

Jara-Acevedo, Maria ;

Schena, Donatella ;

Mayado, Andrea ;

Zamo, Alberto ;

Mollejo, Manuela ;

Sanchez-Lopez, Paula ;

Cabanes, Nieves ;

Orfao, Alberto ;

Escribano, Luis .

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, 2014, 133 (02) :520-+

[2]

[Anonymous], 2016, NIH CLIN RES TRIALS

[3]

[Anonymous], 2018, WHO Classification of Tumours of Endocrine Organs

[4] KIT mutation analysis in mast cell neoplasms: recommendations of the European Competence Network on Mastocytosis [J].

Arock, M. ;

Sotlar, K. ;

Akin, C. ;

Broesby-Olsen, S. ;

Hoermann, G. ;

Escribano, L. ;

Kristensen, T. K. ;

Kluin-Nelemans, H. C. ;

Hermine, O. ;

Dubreuil, P. ;

Sperr, W. R. ;

Hartmann, K. ;

Gotlib, J. ;

Cross, N. C. P. ;

Haferlach, T. ;

Garcia-Montero, A. ;

Orfao, A. ;

Schwaab, J. ;

Triggiani, M. ;

Horny, H-P ;

Metcalfe, D. D. ;

Reiter, A. ;

Valent, P. .

LEUKEMIA, 2015, 29 (06) :1223-1232

[5] Multidisciplinary Management of Mastocytosis: Nordic Expert Group Consensus [J].

Broesby-Olsen, Sigurd ;

Dybedal, Ingunn ;

Gulen, Theo ;

Kristensen, Thomas K. ;

Moller, Michael B. ;

Ackermann, Leena ;

Saaf, Maria ;

Karlsson, Maria ;

Agertoft, Lone ;

Brixen, Kim ;

Hermann, Pernille ;

Stylianou, Eva ;

Mortz, Charlotte G. ;

Torfing, Trine ;

Havelund, Troels ;

Sander, Birgitta ;

Bergstrom, Anna ;

Bendix, Marie ;

Garvey, Lene H. ;

Weis Bjerrum, Ole ;

Valent, Peter ;

Bindslev-Jensen, Carsten ;

Nilsson, Gunnar ;

Vestergaard, Hanne ;

Hagglund, Hans .

ACTA DERMATO-VENEREOLOGICA, 2016, 96 (05) :602-+

[6] Epidemiology of systemic mastocytosis in Denmark [J].

Cohen, Sarah S. ;

Skovbo, Stine ;

Vestergaard, Hanne ;

Kristensen, Thomas ;

Moller, Michael ;

Bindslev-Jensen, Carsten ;

Fryzek, Jon P. ;

Broesby-Olsen, Sigurd .

BRITISH JOURNAL OF HAEMATOLOGY, 2014, 166 (04) :521-528

[7] Cutaneous manifestations in patients with mastocytosis: Consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology [J].

Hartmann, Karin ;

Escribano, Luis ;

Grattan, Clive ;

Brockow, Knut ;

Carter, Melody C. ;

Alvarez-Twose, Ivan ;

Matito, Almudena ;

Broesby-Olsen, Sigurd ;

Siebenhaar, Frank ;

Lange, Magdalena ;

Niedoszytko, Marek ;

Castells, Mariana ;

Oude Elberink, Joanna N. G. ;

Bonadonna, Patrizia ;

Zanotti, Roberta ;

Hornick, Jason L. ;

Torrelo, Antonio ;

Grabbe, Juergen ;

Rabenhorst, Anja ;

Nedoszytko, Boguslaw ;

Butterfield, Joseph H. ;

Gotlib, Jason ;

Reiter, Andreas ;

Radia, Deepti ;

Hermine, Olivier ;

Sotlar, Karl ;

George, Tracy I. ;

Kristensen, Thomas K. ;

Kluin-Nelemans, Hanneke C. ;

Yavuz, Selim ;

Hagglund, Hans ;

Sperr, Wolfgang R. ;

Schwartz, Lawrence B. ;

Triggiani, Massimo ;

Maurer, Marcus ;

Nilsson, Gunnar ;

Horny, Hans-Peter ;

Arock, Michel ;

Orfao, Alberto ;

Metcalfe, Dean D. ;

Akin, Cem ;

Valent, Peter .

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, 2016, 137 (01) :35-45

[8] Serum tryptase and SCORMA (SCORing MAstocytosis) Index as disease severity parameters in childhood and adult cutaneous mastocytosis [J].

Heide, R. ;

van Doorn, K. ;

Mulder, P. G. ;

van Toorenenbergen, A. W. ;

Beishuizen, A. ;

de Groot, H. ;

Tank, B. ;

Oranje, A. P. .

CLINICAL AND EXPERIMENTAL DERMATOLOGY, 2009, 34 (04) :462-468

[9] Impact of centralized evaluation of bone marrow histology in systemic mastocytosis [J].

Jawhar, Mohamad ;

Schwaab, Juliana ;

Horny, Hans-Peter ;

Sotlar, Karl ;

Naumann, Nicole ;

Fabarius, Alice ;

Valent, Peter ;

Cross, Nicholas C. P. ;

Hofmann, Wolf-Karsten ;

Metzgeroth, Georgia ;

Reiter, Andreas .

EUROPEAN JOURNAL OF CLINICAL INVESTIGATION, 2016, 46 (05) :392-397

[10] Sensitive KIT D816V mutation analysis of blood as a diagnostic test in mastocytosis [J].

Kristensen, Thomas ;

Vestergaard, Hanne ;

Bindslev-Jensen, Carsten ;

Moller, Michael Boe ;

Broesby-Olsen, Sigurd .

AMERICAN JOURNAL OF HEMATOLOGY, 2014, 89 (05) :493-498

← 1 2 3 →