2-CdA responses (symptoms + infiltration) according to mastocytosis subtypes. ASM, aggressive systemic mastocytosis; CM, cutaneous mastocytosis; ISM, indolent systemic mastocytosis; MCL, mast cell leukemia; MIS, mastocytosis in the skin; MR, major response; N, number of patients evaluated for 2-CdA response in each WHO mastocytosis subtype; ORR, overall response rate; SM (ISM, SSM, ASM)-AHNMD, systemic mastocytosis with an associated clonal hematological non–mast cell lineage disease; SSM, smoldering systemic mastocytosis.