Richter transformation: histologically confirmed transformation of CLL/SLL to aggressive...

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ID	`RF-RICHTER-TRANSFORMATION`
Tipo	Sinal de alerta
Estado	reviewed 2026-04-27 \| pending_clinical_signoff
Doenças	`DIS-CLL`
Fontes	`SRC-ESMO-CLL-2024` `SRC-NCCN-BCELL-2025`

Origem da bandeira vermelha

Definição	Richter transformation: histologically confirmed transformation of CLL/SLL to aggressive lymphoma — DLBCL-type (most common, ~90%) or Hodgkin-type (~10%). Triggers immediate switch from CLL-directed therapy (BTKi, BCL2i, CIT) to DLBCL-style chemoimmunotherapy (R-CHOP, R-EPOCH; outcomes poor → consider clinical trial, CAR-T post-induction, allogeneic SCT in fit).
Direcção clínica	intensificar
Categoria	transformação-progressão

Lógica de Desencadeamento

{
  "any_of": [
    {
      "finding": "richter_transformation",
      "value": true
    },
    {
      "finding": "cll_to_dlbcl_transformation",
      "value": true
    },
    {
      "finding": "cll_to_hodgkin_transformation",
      "value": true
    }
  ],
  "type": "composite_score"
}

Notas

Incidence 5-10% over CLL course; risk factors include unmutated IGHV, TP53 disruption, NOTCH1, MYC alterations, prior fludarabine. Clonally- related Richter (>80%) has worse OS (median 8-12 months) than clonally-unrelated (de novo DLBCL in CLL patient, 2-year OS 60%). Treatment paradigm: R-CHOP induction (CR 20-30%), consolidate with alloSCT or CAR-T (TRANSCEND-CLL-004 includes Richter cohort, ORR ~60%); BTKi-Richter — pirtobrutinib + venetoclax + R-CHOP under investigation (Mato), pembrolizumab + ibrutinib (Ding 2017) shows responses; Hodgkin-variant Richter — ABVD/AVD ± brentuximab. PET-CT guided biopsy of FDG-avid lesion (SUVmax >5) recommended in CLL patients with rapid clinical change.

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