Recent studies in SM patients show the presence of SRSF2 mutations in a variable percentage of cases ranging from <1% of Non-AdvSM cases to around one third of AdvSM patients (Table 5), being one of the most frequently mutated genes in SM, particularly in SM-AHN cases [10,11,12,13,29,32,46,50,68,80,87,97]. This evidence concerns the gene SRSF2 and systemic mastocytosis.