Response and OS depending on various baseline and on-treatment parameters. Response rates (A) and OS (B) in 35 midostaurin-treated advSM patients according to various baseline parameters. Only the mutational status in the SRSF2/ASXL1/RUNX1 (S/A/R) gene panel was a significant parameter for prediction of response and OS. (C) On-treatment response parameters in 28 patients at month 6: significant reductions of serum tryptase, AP, KIT D816V EAB, and any response according to Valent criteria were identified as favorable prognostic parameters concerning OS. In multivariate analysis, only a KIT D816V EAB reduction <25% remained an independent risk factor for poor OS. Hb, hemoglobin; PLT, platelets. *, P values refer to Fisher’s exact tests; **, P values refer to the log-rank tests. ^a, MCL patients with and without AHN; ^b, KIT D816V in PB; ^c, AP reduction ≥50% or normalization; ^d, Cheson criteria for transfusions; ^e, response criteria according to Valent.¹⁵