Acute Promyelocytic Leukemia (PML-RARA)
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-APL |
|---|---|
| Type | Disease |
| Aliases | AML M3 (legacy FAB)APLAcute Promyelocytic LeukemiaPromyelocytic leukemiaГострий промієлоцитарний лейкоз (PML-RARA) |
| Status | reviewed 2026-04-25 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-APL0406-LOCOCO-2013 SRC-ELN-APL-2019 SRC-NCCN-AML-2025 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 9866/3 |
|---|---|
| icd_o_3_topography | ["C42.1"] |
| icd_10 | C92.4 |
| who_classification | Acute promyelocytic leukemia with PML::RARA fusion (per WHO 5th ed.) |
Notes
Separated from DIS-AML because clinical logic is fundamentally different: (a) emergency ATRA initiation on suspicion (within hours of presentation), (b) DIC management is the dominant early task, (c) chemotherapy-free ATRA + ATO cures low-risk patients (APL0406 / LO-CoCo), (d) high-risk uses ATRA + ATO + idarubicin (or classic AIDA). NCCN and ELN both treat APL as a separate chapter — we mirror that. CHARTER §15.2 C2 — emergency initiation is partially time-critical (outside non-device CDS carve-out for the first dose); our scope is workup + plan AFTER initial stabilization. STUB — requires clinical co-lead signoff before publication. Myeloid expansion (heme).
Used By
Algorithms
ALGO-APL-1L- ALGO-APL-1LALGO-APL-2L- ALGO-APL-2L
Disease
DIS-AML- Acute Myeloid Leukemia (non-APL)
Indication
IND-APL-1L-ATRA-ATO- IND-APL-1L-ATRA-ATOIND-APL-1L-ATRA-ATO-IDA- IND-APL-1L-ATRA-ATO-IDAIND-APL-RELAPSED-GEMTUZUMAB- IND-APL-RELAPSED-GEMTUZUMABIND-APL-SALVAGE-ATRA-ATO- IND-APL-SALVAGE-ATRA-ATO
Questionnaires
QUEST-APL-1L-STUB- Acute Promyelocytic Leukemia — first line
Red flag
RF-APL-EMERGENCY-DIC- APL with active disseminated intravascular coagulation (DIC): fibrinogen <150 mg/dL, prol...RF-APL-FRAILTY-AGE- APL patient elderly (age ≥70) or frail (ECOG ≥2 with comorbidities) — chemo-free ATRA + A...RF-APL-HIGH-RISK-BIOLOGY- APL high-risk by Sanz score: presenting WBC >10 K/μL — requires anthracycline addition (i...RF-APL-INFECTION-SCREENING- APL patient with positive HBV / HCV / HIV serology, latent TB, or active uncontrolled inf...RF-APL-ORGAN-DYSFUNCTION- APL with cardiac comorbidity making ATO problematic: baseline QTc >500 ms, uncorrected hy...RF-APL-TRANSFORMATION-PROGRESSION- APL differentiation syndrome (formerly RA-syndrome): fever, dyspnea, weight gain ≥5 kg, p...RF-SANZ-HIGH-RISK- APL with Sanz high risk — WBC >10 ×10^9/L at diagnosis — supports ATRA+ATO+idarubicin (or...RF-SANZ-INTERMEDIATE- APL with Sanz intermediate risk — WBC ≤10 ×10^9/L AND platelets ≤40 ×10^9/L — eligible fo...RF-SANZ-LOW-RISK- APL with Sanz low risk — WBC ≤10 ×10^9/L AND platelets >40 ×10^9/L — supports ATRA+ATO ch...
Sources
SRC-APL-RELAPSE-LENGFELDER-2017- Outcomes of patients with relapsed acute promyelocytic leukemia treated with all-trans re...SRC-APL0406-LOCOCO-2013- Retinoic acid and arsenic trioxide for acute promyelocytic leukemiaSRC-ELN-AML-2022- Diagnosis and management of AML in adults: 2022 ELN recommendations from an international...SRC-ELN-APL-2019- Management of acute promyelocytic leukemia: updated recommendations from an expert panel...SRC-ESMO-AML-2020- Acute myeloid leukaemia in adult patients: ESMO Clinical Practice Guidelines for diagnosi...SRC-MYLOFRANCE-ARIBI-2007- Combination therapy with arsenic trioxide for relapsed acute promyelocytic leukemia