OnCo

Systemic mastocytosis: lines of therapy

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4 standard-of-care settings across 3 lines and 1 biomarker subgroup. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.

LineAll comers
Early / localised1
Advanced, first line2
Other settings1

Early / localised

SubgroupSettingApproachProducts and trialsEvidence
All comersIndolent SM, symptomaticH1 and H2 antihistamines, cromolyn, leukotriene antagonists, omalizumab for anaphylaxis, epinephrine autoinjector, bone protection; avapritinib 25 mg daily for moderate to severe symptoms uncontrolled by these (PIONEER).62

Advanced, first line

SubgroupSettingApproachProducts and trialsEvidence
All comersAdvanced SM, first lineAvapritinib 200 mg daily (platelets above 50 x 10^9/L) as preferred agent; midostaurin as alternative or where platelets are low.97
All comersAdvanced SM, subsequent linesSwitch between avapritinib and midostaurin; cladribine; clinical trials (bezuclastinib, elenestinib); allogeneic transplant for mast cell leukaemia or high-risk SM-AHN.87

Other settings

SubgroupSettingApproachProducts and trialsEvidence
All comersSM-AHNTreat the dominant component: KIT inhibitor for mast cell burden plus the standard therapy for the associated CMML, MDS or AML (hypomethylating agents, intensive chemotherapy, transplant).89

Lines and subgroups are parsed from the setting text of each standard-of-care row and can misclassify an unusual phrasing; the row’s own setting is always shown. Guideline chips reflect the NCCN category and ESMO-MCBS grade recorded on the cancer page, checked on its stated date. Not medical advice.