In ASM, treatments interfering with MC proliferation and survival are used. Tyrosine kinase inhibitors (TKIs), chemotherapy, and allogenic stem cell transplantation (allo-SCT) are considered the best therapeutic options for ASM, including MCL
[21][22][23]. Imatinib and Nilotinib demonstrate effect against wild-type KIT and a limited activity against KIT D816V; however, there are reports of response to these drugs in patients with atypical KIT mutations
[4][24]. Midostaurin, a TKI with activity against KIT D816V, represents the only approved therapy for advanced SM, including MCL
[25]. In advanced SM, most responses with Midostaurin are only partial and not sustained. Other medications potentially effective for SM patients are under investigation. Recent and promising results have been obtained with Avapritinib, a KIT and PDGFRA inhibitor
[26][27]. Interferon alpha and cladribrine have also been used
[28][29]. Allo-SCT needs to be considered in young and otherwise healthy patients with ASM, as this is the only option for a sustained response
[30]. Prior to SCT, therapies such as either midostaurin or cladribrine should be used to bring the patients to the best response.