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CLL with clinical / biochemical features concerning for Richter transformation (to DLBCL...

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IDRF-CLL-TRANSFORMATION-PROGRESSION
TypeSignal d’alerte
Étatreviewed 2026-04-27 | pending_clinical_signoff
MaladiesDIS-CLL
SourcesSRC-ESMO-CLL-2024 SRC-NCCN-BCELL-2025

Origine du drapeau rouge

DéfinitionCLL with clinical / biochemical features concerning for Richter transformation (to DLBCL or rarely Hodgkin variant): rapid LDH rise, asymmetric / rapidly enlarging nodal or extranodal mass, PET-CT SUVmax >10, B-symptoms in previously asymptomatic patient, or hypercalcemia. Mandates urgent excisional biopsy of PET-avid lesion.
Direction cliniqueintensifier
Catégorietransformation-progression

Logique de déclenchement

{
  "any_of": [
    {
      "finding": "ldh_doubled_in_weeks",
      "value": true
    },
    {
      "finding": "rapid_progression",
      "value": true
    },
    {
      "comparator": ">",
      "finding": "pet_suvmax",
      "threshold": 10
    },
    {
      "finding": "richter_suspect",
      "value": true
    },
    {
      "finding": "biopsy_shows_dlbcl",
      "value": true
    },
    {
      "finding": "new_b_symptoms",
      "value": true
    }
  ],
  "type": "composite_clinical"
}

Notes

Richter transformation occurs in 5-10% CLL lifetime risk; clonally related to underlying CLL ~80% (poor prognosis, median OS <12 mo on R-CHOP) vs clonally unrelated ~20% (de novo DLBCL biology, R-CHOP curable). PET-CT SUVmax >10 has ~70% sensitivity/specificity for Richter — mandates biopsy of hottest lesion (excisional preferred over core, FDG-avid heterogeneity common). Confirmed Richter: R-CHOP induction; consider acalabrutinib + rituximab + R-CHOP combinations on trial; alloHCT consolidation in fit. Hodgkin-variant Richter (~1%): treat as cHL (ABVD or A+AVD).

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Indication