Clinical presentation and management practice of systemic mastocytosis. A survey on 460 Italian patients

Lisa Pieri, Patrizia Bonadonna, Chiara Elena, Cristina Papayannidis, Federica Grifoni, M. Rondoni, Stefania Girlanda, Marina Mauro, Diomira Magliacane, Elena Elli, Maria Loredana Iorno, Fabio Almerigogna, Federica Scarfì, Roberto Salerno, Tiziana Fanelli, F. Gesullo, Giuditta Corbizi Fattori, Massimiliano Bonifacio, Omar Perbellini, Anna Artuso & 13 others S. Soverini, Caterina De Benedittis, Simona Muratori, Valerio Pravettoni, Vittoria Cova, Gabriele Cortellini, Fabio Ciceri, Agostino Cortelezzi, G. Martinelli, M. Triggiani, Serena Merante, Alessandro M. Vannucchi, R. Zanotti

Research output: Contribution to journalArticle

Abstract

Systemic mastocytosis is a rare heterogeneous myeloproliferative neoplasm characterized by abnormal proliferation and activation of mast cells. We describe a large multicentre series of 460 adult patients with systemic mastocytosis, with a diagnosis based on WHO 2008 criteria, in a “real-life” setting of ten Italian centers with dedicated multidisciplinary programs. We included indolent forms with (n = 255) and without (n = 165) skin lesions, smouldering (n = 20), aggressive (n = 28), associated with other hematological diseases mastocytosis (n = 21) and mast cell leukemia (n = 1). This series was uniquely characterized by a substantial proportion of patients with low burden of neoplastic mast cells; notably, 38% of cases were diagnosed using only minor diagnostic criteria according to WHO 2008 classification, underlying the feasibility of early diagnosis where all diagnostic approaches are made available. This has particular clinical relevance for prevention of anaphylaxis manifestations, that were typically associated with indolent forms. In multivariate analysis, the most important features associated with shortened overall survival were disease subtype and age at diagnosis >60 years. Disease progression was correlated with mastocytosis subtype and thrombocytopenia. As many as 32% of patients with aggressive mastocytosis suffered from early evolution into acute leukemia. Overall, this study provides novel information about diagnostic approaches and current presentation of patients with SM and underlines the importance of networks and specialized centers to facilitate early diagnosis and prevent disease-associated manifestations. Am. J. Hematol. 91:692–699, 2016. © 2016 Wiley Periodicals, Inc.

Original languageEnglish
Pages (from-to)692-699
Number of pages8
JournalAmerican Journal of Hematology
Volume91
Issue number7
DOIs
Publication statusPublished - Jul 1 2016

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Systemic Mastocytosis
Practice Management
Mastocytosis
Mast Cells
Early Diagnosis
Mast-Cell Leukemia
Hematologic Diseases
Anaphylaxis
Thrombocytopenia
Disease Progression
Leukemia
Multivariate Analysis
Skin
Survival
Surveys and Questionnaires
Neoplasms

ASJC Scopus subject areas

  • Hematology

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Clinical presentation and management practice of systemic mastocytosis. A survey on 460 Italian patients. / Pieri, Lisa; Bonadonna, Patrizia; Elena, Chiara; Papayannidis, Cristina; Grifoni, Federica; Rondoni, M.; Girlanda, Stefania; Mauro, Marina; Magliacane, Diomira; Elli, Elena; Iorno, Maria Loredana; Almerigogna, Fabio; Scarfì, Federica; Salerno, Roberto; Fanelli, Tiziana; Gesullo, F.; Corbizi Fattori, Giuditta; Bonifacio, Massimiliano; Perbellini, Omar; Artuso, Anna; Soverini, S.; De Benedittis, Caterina; Muratori, Simona; Pravettoni, Valerio; Cova, Vittoria; Cortellini, Gabriele; Ciceri, Fabio; Cortelezzi, Agostino; Martinelli, G.; Triggiani, M.; Merante, Serena; Vannucchi, Alessandro M.; Zanotti, R.

In: American Journal of Hematology, Vol. 91, No. 7, 01.07.2016, p. 692-699.

Research output: Contribution to journalArticle

Pieri, L, Bonadonna, P, Elena, C, Papayannidis, C, Grifoni, F, Rondoni, M, Girlanda, S, Mauro, M, Magliacane, D, Elli, E, Iorno, ML, Almerigogna, F, Scarfì, F, Salerno, R, Fanelli, T, Gesullo, F, Corbizi Fattori, G, Bonifacio, M, Perbellini, O, Artuso, A, Soverini, S, De Benedittis, C, Muratori, S, Pravettoni, V, Cova, V, Cortellini, G, Ciceri, F, Cortelezzi, A, Martinelli, G, Triggiani, M, Merante, S, Vannucchi, AM & Zanotti, R 2016, 'Clinical presentation and management practice of systemic mastocytosis. A survey on 460 Italian patients', American Journal of Hematology, vol. 91, no. 7, pp. 692-699. https://doi.org/10.1002/ajh.24382
Pieri, Lisa ; Bonadonna, Patrizia ; Elena, Chiara ; Papayannidis, Cristina ; Grifoni, Federica ; Rondoni, M. ; Girlanda, Stefania ; Mauro, Marina ; Magliacane, Diomira ; Elli, Elena ; Iorno, Maria Loredana ; Almerigogna, Fabio ; Scarfì, Federica ; Salerno, Roberto ; Fanelli, Tiziana ; Gesullo, F. ; Corbizi Fattori, Giuditta ; Bonifacio, Massimiliano ; Perbellini, Omar ; Artuso, Anna ; Soverini, S. ; De Benedittis, Caterina ; Muratori, Simona ; Pravettoni, Valerio ; Cova, Vittoria ; Cortellini, Gabriele ; Ciceri, Fabio ; Cortelezzi, Agostino ; Martinelli, G. ; Triggiani, M. ; Merante, Serena ; Vannucchi, Alessandro M. ; Zanotti, R. / Clinical presentation and management practice of systemic mastocytosis. A survey on 460 Italian patients. In: American Journal of Hematology. 2016 ; Vol. 91, No. 7. pp. 692-699.
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T1 - Clinical presentation and management practice of systemic mastocytosis. A survey on 460 Italian patients

AU - Pieri, Lisa

AU - Bonadonna, Patrizia

AU - Elena, Chiara

AU - Papayannidis, Cristina

AU - Grifoni, Federica

AU - Rondoni, M.

AU - Girlanda, Stefania

AU - Mauro, Marina

AU - Magliacane, Diomira

AU - Elli, Elena

AU - Iorno, Maria Loredana

AU - Almerigogna, Fabio

AU - Scarfì, Federica

AU - Salerno, Roberto

AU - Fanelli, Tiziana

AU - Gesullo, F.

AU - Corbizi Fattori, Giuditta

AU - Bonifacio, Massimiliano

AU - Perbellini, Omar

AU - Artuso, Anna

AU - Soverini, S.

AU - De Benedittis, Caterina

AU - Muratori, Simona

AU - Pravettoni, Valerio

AU - Cova, Vittoria

AU - Cortellini, Gabriele

AU - Ciceri, Fabio

AU - Cortelezzi, Agostino

AU - Martinelli, G.

AU - Triggiani, M.

AU - Merante, Serena

AU - Vannucchi, Alessandro M.

AU - Zanotti, R.

PY - 2016/7/1

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N2 - Systemic mastocytosis is a rare heterogeneous myeloproliferative neoplasm characterized by abnormal proliferation and activation of mast cells. We describe a large multicentre series of 460 adult patients with systemic mastocytosis, with a diagnosis based on WHO 2008 criteria, in a “real-life” setting of ten Italian centers with dedicated multidisciplinary programs. We included indolent forms with (n = 255) and without (n = 165) skin lesions, smouldering (n = 20), aggressive (n = 28), associated with other hematological diseases mastocytosis (n = 21) and mast cell leukemia (n = 1). This series was uniquely characterized by a substantial proportion of patients with low burden of neoplastic mast cells; notably, 38% of cases were diagnosed using only minor diagnostic criteria according to WHO 2008 classification, underlying the feasibility of early diagnosis where all diagnostic approaches are made available. This has particular clinical relevance for prevention of anaphylaxis manifestations, that were typically associated with indolent forms. In multivariate analysis, the most important features associated with shortened overall survival were disease subtype and age at diagnosis >60 years. Disease progression was correlated with mastocytosis subtype and thrombocytopenia. As many as 32% of patients with aggressive mastocytosis suffered from early evolution into acute leukemia. Overall, this study provides novel information about diagnostic approaches and current presentation of patients with SM and underlines the importance of networks and specialized centers to facilitate early diagnosis and prevent disease-associated manifestations. Am. J. Hematol. 91:692–699, 2016. © 2016 Wiley Periodicals, Inc.

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