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APL differentiation syndrome (formerly RA-syndrome): fever, dyspnea, weight gain ≥5 kg, p...

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

IDRF-APL-TRANSFORMATION-PROGRESSION
TypeRed flag
Statusreviewed 2026-04-25 | pending_clinical_signoff
DiseasesDIS-APL
SourcesSRC-ELN-APL-2019 SRC-NCCN-AML-2025

Red Flag Origin

DefinitionAPL differentiation syndrome (formerly RA-syndrome): fever, dyspnea, weight gain ≥5 kg, pleuropericardial effusions, pulmonary infiltrates, hypotension, AKI — typically days 2-21 of induction
Clinical directioninvestigate
Categorytransformation-progression

Trigger Logic

{
  "any_of": [
    {
      "finding": "differentiation_syndrome_active",
      "value": true
    },
    {
      "finding": "rising_wbc_on_atra",
      "value": true
    },
    {
      "comparator": ">",
      "finding": "wbc_rise_during_induction",
      "threshold": 10
    },
    {
      "all_of": [
        {
          "finding": "fever_unexplained",
          "value": true
        },
        {
          "finding": "new_dyspnea",
          "value": true
        },
        {
          "comparator": ">=",
          "finding": "weight_gain_kg",
          "threshold": 5
        }
      ]
    }
  ],
  "type": "symptom_composite"
}

Notes

Direction "investigate" — surfaces a monitoring annotation rather than shifting indication. Triggers immediate dexamethasone 10 mg IV BID, consideration of temporary ATRA/ATO hold (per severity), aggressive diuresis, ICU monitoring. Prophylactic steroids (prednisone 0.5 mg/kg or dexamethasone 2.5 mg/m² BID) are recommended for high-risk (WBC >10) and any patient with rising WBC during induction. DS occurs in ~5-25% and is a leading cause of induction mortality if not promptly recognized. STUB — requires clinical co-lead signoff.

Used By

Algorithms

Indications