Systemic mastocytosis (SM) is a clonal disorder with complex manifestations determined by the proliferation and accumulation of mast cells in various organs (mainly in skin and bone marrow) and by the release of soluble mediators. Indolent SM is the more frequent variant of the disease, but its actual prevalence is unknown as the majority of patients suffering from SM are misdiagnosed, particularly when skin lesions are absent. Anaphylactic reactions to Hymenoptera (and less frequently to drugs or foods), idiopatic anaphylaxis, unexplained osteoporosis, or unexplained gastrointestinal symptoms are common manifestations of SM in absence of skin involvement. Therefore, the diagnosis and management of patients with SM require a multidisciplinary approach, which encompasses hematological, allergological, dermatological, rheumatological, and gastroenterological evaluations. A correct and timely diagnosis is required for: a) adequate counseling of patients and their physicians; b) beginning of symptomatic treatment (antimediator therapy); c) prevention of more severe manifestations of the disease (i.e., recurrent anaphylaxis, osteoporosis, bone fractures). In this paper, the epidemiology, classification, diagnostic tools, clinical manifestations, and therapy of SM are reviewed and a guidance on multidisciplinary approach to SM is provided.

Systemic mastocytosis: a muldisciplinary approach (Review) [Lamastocitosi sistemica: Un approccio multidiscliplinare]

ZANOTTI, ROBERTA;BONADONNA, PATRIZIA;BONIFACIO, Massimiliano;ROSSINI, Maurizio;
2012

Abstract

Systemic mastocytosis (SM) is a clonal disorder with complex manifestations determined by the proliferation and accumulation of mast cells in various organs (mainly in skin and bone marrow) and by the release of soluble mediators. Indolent SM is the more frequent variant of the disease, but its actual prevalence is unknown as the majority of patients suffering from SM are misdiagnosed, particularly when skin lesions are absent. Anaphylactic reactions to Hymenoptera (and less frequently to drugs or foods), idiopatic anaphylaxis, unexplained osteoporosis, or unexplained gastrointestinal symptoms are common manifestations of SM in absence of skin involvement. Therefore, the diagnosis and management of patients with SM require a multidisciplinary approach, which encompasses hematological, allergological, dermatological, rheumatological, and gastroenterological evaluations. A correct and timely diagnosis is required for: a) adequate counseling of patients and their physicians; b) beginning of symptomatic treatment (antimediator therapy); c) prevention of more severe manifestations of the disease (i.e., recurrent anaphylaxis, osteoporosis, bone fractures). In this paper, the epidemiology, classification, diagnostic tools, clinical manifestations, and therapy of SM are reviewed and a guidance on multidisciplinary approach to SM is provided.
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11562/435263
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