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Variante histológica blastoide ou pleomórfica do linfoma de células do manto — ~10-15% do LCM em di...

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IDRF-MCL-BLASTOID-VARIANT
TipoSinal de alerta
Estadoreviewed 2026-04-27 | pending_clinical_signoff
DoençasDIS-MCL
FontesSRC-ESMO-MCL-2024 SRC-NCCN-BCELL-2025 SRC-ZUMA-2-WANG-2020

Origem da bandeira vermelha

DefiniçãoBlastoid or pleomorphic histologic variant of mantle cell lymphoma — ~10-15% of MCL at diagnosis, biology aggressive (Ki-67 typically >50%, often co-mutated TP53/CDKN2A), poor response to chemoimmunotherapy (BR, R-CHOP, R-DHAP) with median OS <2 years on cBTKi. ZUMA-2 (Wang NEJM 2020) established brexucabtagene autoleucel (KTE-X19) for R/R MCL post-cBTKi (ORR 91%, CR 68%, mPFS not reached at 24 mo). Routes blastoid/pleomorphic MCL to CAR-T prioritization at 2L (post-cBTKi or post-chemoimmuno failure) over alloHCT or further chemoimmuno.
Direcção clínicaintensificar
Categoriahigh-risk-biology
Algoritmo de deslocamentosALGO-MCL-2L

Lógica de Desencadeamento

{
  "any_of": [
    {
      "finding": "mcl_histology_variant",
      "value": "blastoid"
    },
    {
      "finding": "mcl_histology_variant",
      "value": "pleomorphic"
    },
    {
      "finding": "blastoid_morphology",
      "value": true
    },
    {
      "finding": "pleomorphic_morphology",
      "value": true
    }
  ],
  "type": "biomarker"
}

Notas

Distinguish from RF-MCL-BLASTOID-OR-TP53 — that flag is broader (any high-risk biology including TP53/del(17p)/Ki-67>30%) and routes 1L to BTKi-based; this flag is narrower (histology-defined blastoid/pleomorphic morphology) and routes 2L specifically to brexu-cel CAR-T. Both fire in parallel for blastoid + TP53-mut cases. Brexu-cel manufacture takes ~3 weeks — bridge with cBTKi (acalabrutinib) or pirtobrutinib if cBTKi-pretreated. Eligibility check via RF-CAR-T-ELIGIBLE composite. Lisocabtagene maraleucel (TRANSCEND-NHL-001 MCL cohort) is a second CAR-T option with similar efficacy and lower CRS/ICANS rates. Access UA: CAR-T not domestically available; international referral pathway only.

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Algoritmos

Indicação