Systemic Mastocytosis: An Intriguing Disorder

SM is a rare disorder, but its prevalence might be underestimated. Afar from drafting a complete and exhaustive review on mastocytosis, the aim of this chapter was to remark the relevance of SM. Clinical suspicion is really important and multimodality approaches must be considered to get the right diagnosis. Then, treatment must be personalized for each patient, accordingly with a careful and complete characterization of the disease. Prospectively, two major challenges still have to be faced in SM research: first, the molecular and cellular pathogenesis; second, the definition of new strategies of treatment. About the former goal, we do believe that reporting new cases may be of great usefulness and is needed to better understand the nature of the disorder. In order to this, the case we described may perhaps represent a paradigmatic example. Referring to treatment, several novel agents are under investigation. However, preliminary clinical data seem to suggest that the use of a single drug may be insufficient. That means that a multidrug strategy is needed within a multitarget approach.