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ET transformation to post-ET myelofibrosis (~5-10% over 15 years) or to AML / MDS (rare ~...

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

IDRF-ET-TRANSFORMATION-PROGRESSION
TypeRed flag
Statusreviewed 2026-04-25 | pending_clinical_signoff
DiseasesDIS-ET
SourcesSRC-ESMO-MPN-2015 SRC-NCCN-MPN-2025

Red Flag Origin

DefinitionET transformation to post-ET myelofibrosis (~5-10% over 15 years) or to AML / MDS (rare ~1-5%): rising LDH, new splenomegaly, leukoerythroblastic smear, increasing reticulin fibrosis on trephine, blast appearance — re-stage, switch to MF or AML algorithm
Clinical directionintensify
Categorytransformation-progression
Shifts algorithmALGO-ET-1L

Trigger Logic

{
  "any_of": [
    {
      "finding": "post_et_mf_transformation",
      "value": true
    },
    {
      "finding": "post_et_aml_transformation",
      "value": true
    },
    {
      "comparator": ">=",
      "finding": "blasts_pb_pct",
      "threshold": 5
    },
    {
      "comparator": ">=",
      "finding": "blasts_bm_pct",
      "threshold": 5
    },
    {
      "finding": "rapid_splenomegaly_progression",
      "value": true
    },
    {
      "finding": "leukoerythroblastic_smear",
      "value": true
    }
  ],
  "type": "composite"
}

Notes

Triggers re-route from ET 1L algorithm to PMF algorithm (post-ET MF) or AML algorithm (post-ET AML / MDS). Annual incidence is low; risk rises with disease duration + JAK2-mutation status + exposure to cytoreduction (especially anagrelide vs HU per PT-1). STUB — requires clinical co-lead signoff.

Used By

Algorithms

Indications