Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia): lines of therapy
5 standard-of-care settings across 5 lines and 2 biomarker subgroups. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.
| Line | All comers | Risk group |
|---|---|---|
| Screening, prevention and diagnosis | 1 | · |
| Early / localised | · | 1 |
| Advanced, first line | 1 | · |
| Second line | 1 | · |
| Other settings | 1 | · |
Screening, prevention and diagnosis
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Diagnosis and risk assessment | Bone marrow biopsy and aspirate, KIT D816V allele burden, myeloid mutation panel, tryptase, imaging for organomegaly and bone lesions, MARS or IPSM score. | NCCN Guidelines: Systemic Mastocytosis | 95 |
Early / localised
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| Risk group | Fit patients, especially mast cell leukaemia or high-risk SM-AHN | Allogeneic haematopoietic cell transplantation after response to a KIT inhibitor. | NCCN Guidelines: Systemic Mastocytosis | 92 |
Advanced, first line
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | First line | Avapritinib 200 mg daily when platelets are 50 x 10^9/L or more (EXPLORER, PATHFINDER, approved 2021); midostaurin as the alternative (approved 2017). | NCCN Guidelines: Systemic Mastocytosis | 97 |
Second line
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Relapsed or intolerant | Switch between avapritinib and midostaurin; cladribine; interferon alfa; bezuclastinib in the Apex trial. | NCCN Guidelines: Systemic Mastocytosis | 43 |
Other settings
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Associated haematological neoplasm | Treat the neoplasm on its own merits (azacitidine for CMML or MDS, intensive chemotherapy for AML) alongside or after KIT inhibition. | NCCN Guidelines: Systemic Mastocytosis | 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.