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 language | English |
|---|---|
| Pages (from-to) | 692-699 |
| Number of pages | 8 |
| Journal | American Journal of Hematology |
| Volume | 91 |
| Issue number | 7 |
| DOIs | |
| Publication status | Published - Jul 1 2016 |
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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 journal › Article
}
TY - JOUR
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
Y1 - 2016/7/1
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.
AB - 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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U2 - 10.1002/ajh.24382
DO - 10.1002/ajh.24382
M3 - Article
VL - 91
SP - 692
EP - 699
JO - American Journal of Hematology
JF - American Journal of Hematology
SN - 0361-8609
IS - 7
ER -