Mastocytosis with progression to mast cell leukemia (MCL) or sarcomatous transformation:...

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

ID	`RF-MASTOCYTOSIS-TRANSFORMATION-PROGRESSION`
Type	Red flag
Status	reviewed 2026-04-27 \| pending_clinical_signoff
Diseases	`DIS-MASTOCYTOSIS`
Sources	`SRC-NCCN-SM-2025` `SRC-WHO-LNSC-2023`

Red Flag Origin

Definition	Mastocytosis with progression to mast cell leukemia (MCL) or sarcomatous transformation: rising mast cell burden in BM ≥20%, circulating mast cells ≥10% of WBC, or extracutaneous solid mast cell tumor
Clinical direction	intensify
Category	transformation-progression
Shifts algorithm	ALGO-ADVSM-1L

Trigger Logic

{
  "any_of": [
    {
      "comparator": ">=",
      "finding": "bm_mast_cells_percent",
      "threshold": 20
    },
    {
      "comparator": ">=",
      "finding": "pb_mast_cells_percent",
      "threshold": 10
    },
    {
      "finding": "mast_cell_sarcoma_new",
      "value": true
    },
    {
      "finding": "rapid_burden_progression",
      "value": true
    }
  ],
  "type": "composite"
}

Notes

MCL has dismal prognosis (median OS <1 year historically). Per NCCN-SM: avapritinib is preferred for MCL (PATHFINDER showed responses in MCL subset); allogeneic HCT considered for fit patients in remission. Direction INTENSIFY → cytoreduction + consider HCT. STUB — requires clinical co-lead signoff.

Used By

No reverse references found in the YAML corpus.