biochimica clinica

it_IT Italian
it_IT Italian en_US English

RASSEGNE - Reviews

Volume:

Biochimica Clinica 2012; 36(3) 158-170

Pubblicato on-line:

DOI:

Scarica in PDF:
Autenticazione richiesta

La mastocitosi sistemica: un approccio multidiscliplinare

AUTORI

Roberta Zanotti1,2, Patrizia Bonadonna1,3, Massimiliano Bonifacio1,2, Beatrice Caruso1,4, Chiara Colato5, Giovanna De Matteis6, Omar Perbellini1, Maurizio Rossini1,7, Chiara Sabbadini8, Donatella Schena1,8, Morena Tebaldi9, Alberto Zamò5
1Ambulatorio Integrato per lo Studio della Mastocitosi, 2Unità Operativa di Ematologia, 3Servizio di Allergologia, 4Laboratorio Analisi, Dipartimento Ospedaliero, 5Anatomia Patologica Dipartimento Universitario, 6Laboratorio Analisi Dipartimento Universitario, 7Unità Operativa di Reumatologia, 8Unità Operativa di Dermatologia, 9Unità Operativa di Gastroenterologia ed Endoscopia Digestiva, Azienda Ospedaliera Universitaria Integrata di Verona

ABSTRACT

Systemic mastocytosis: a muldisciplinary approach

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.

BIBLIOGRAFIA