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ALCL primary-refractory disease (no CR after 4 cycles CHP-Bv) OR early relapse <12 months...

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

IDRF-ALCL-TRANSFORMATION-PROGRESSION
TypeRed flag
Statusreviewed 2026-04-27 | pending_clinical_signoff
DiseasesDIS-ALCL
SourcesSRC-ESMO-PTCL-2024 SRC-NCCN-BCELL-2025

Red Flag Origin

DefinitionALCL primary-refractory disease (no CR after 4 cycles CHP-Bv) OR early relapse <12 months post-induction OR ALK-negative ALCL with DUSP22-WT (poor prognostic subset) — routes to salvage chemotherapy (ICE / DHAP / GEM-P) with intent to consolidate with autoSCT or alloSCT.
Clinical directionintensify
Categorytransformation-progression

Trigger Logic

{
  "any_of": [
    {
      "finding": "alcl_primary_refractory",
      "value": true
    },
    {
      "finding": "alcl_early_relapse_lt_12mo",
      "value": true
    },
    {
      "all_of": [
        {
          "finding": "alk_status",
          "value": "negative"
        },
        {
          "finding": "dusp22_rearrangement",
          "value": "negative"
        }
      ]
    },
    {
      "finding": "rapid_progression",
      "value": true
    }
  ],
  "type": "composite_clinical"
}

Notes

ALK+ ALCL has 70-90% 5-year OS on CHP-Bv; ALK– more heterogeneous. Within ALK–, DUSP22-rearranged subset behaves favorably (~90% 5y OS), while DUSP22-WT/TP63-WT ALK– is poor (40-50% 5y OS) — this drives the prognostic stratification that intensifies upfront consolidation. Primary-refractory or early-relapse ALCL: brentuximab vedotin monotherapy is highly active (50-60% ORR per pivotal Pro et al., J Clin Oncol 2017) for those who did not receive it 1L; otherwise combination salvage + transplant.

Used By

No reverse references found in the YAML corpus.