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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
TipoSinal de alerta
Estadoreviewed 2026-04-27 | pending_clinical_signoff
DoençasDIS-MASTOCYTOSIS
FontesSRC-NCCN-SM-2025 SRC-WHO-LNSC-2023

Origem da bandeira vermelha

DefiniçãoAggressive 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
Direcção clínicaintensificar
Categoriahigh-risk-biology

Lógica de Desencadeamento

{
  "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"
}

Notas

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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