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Aggressive systemic mastocytosis variant by WHO 5th ed.: SM with associated hematologic n...

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IDRF-MASTOCYTOSIS-HIGH-RISK-BIOLOGY
TypeSignal d’alerte
Étatreviewed 2026-04-27 | pending_clinical_signoff
MaladiesDIS-MASTOCYTOSIS
SourcesSRC-NCCN-SM-2025 SRC-WHO-LNSC-2023

Origine du drapeau rouge

DéfinitionAggressive systemic mastocytosis variant by WHO 5th ed.: SM with associated hematologic neoplasm (SM-AHN), aggressive SM (ASM), mast cell leukemia (MCL ≥10% mast cells in PB or ≥20% in BM), mast cell sarcoma; KIT D816V detected
Direction cliniqueintensifier
Catégoriehigh-risk-biology

Logique de déclenchement

{
  "any_of": [
    {
      "finding": "sm_ahn_diagnosis",
      "value": true
    },
    {
      "finding": "asm_diagnosis",
      "value": true
    },
    {
      "finding": "mast_cell_leukemia",
      "value": true
    },
    {
      "finding": "mast_cell_sarcoma",
      "value": true
    },
    {
      "finding": "kit_d816v_positive",
      "value": true
    }
  ],
  "type": "composite"
}

Notes

KIT D816V is present in ≥90% of advSM and is the actionable target for midostaurin (RATIFY/PATHWAY studies) and avapritinib (PATHFINDER, EXPLORER). AHN component (most often myeloid neoplasm — CMML, MDS, MPN-U) is treated separately per its own algorithm AND with KIT-directed therapy. Direction INTENSIFY → KIT-targeted therapy + AHN-directed regimen. STUB — requires clinical co-lead signoff.

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