Leucemia promielocítica aguda (LMP-RARA)
Visão determinística da entidade fonte YAML. A autoridade clínica permanece com os IDs de fonte citados e estado de assinatura do revisor.
| ID | DIS-APL |
|---|---|
| Tipo | Doença |
| Alegais | AML M3 (legado FAB)APLLeucemia promielocítica agudaLeucemia promielocíticaГострий промієлоцитарний лейкоз (PML-RARA) |
| Estado | reviewed 2026-04-25 | pending_clinical_signoff |
| Doenças | Nenhuma declaração |
| Fontes | 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.) |
Notas
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).
Usado por
Algoritmos
ALGO-APL-1L- ALGO-APL-1LALGO-APL-2L- ALGO-APL-2L
Doença
DIS-AML- Acute Myeloid Leukemia (non-APL)
Indicação
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
Questionários
QUEST-APL-1L-STUB- Leucemia Promielocítica Aguda — primeira linha
Sinal de alerta
RF-APL-EMERGENCY-DIC- APL com coagulação intravascular disseminada ativa (DIC): fibrinogênio < 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 de alto risco pela pontuação de Sanz: apresentar WBC >10 K/μL — requer adição de antraciclina (i...RF-APL-INFECTION-SCREENING- APL patient with positive HBV / HCV / HIV serology, latent TB, or active uncontrolled inf...RF-APL-ORGAN-DYSFUNCTION- APL com comorbidade cardíaca tornando problemático o ATO: QTc basal >500 ms, h...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 com risco intermédio de Sanz — WBC ≤10 ×10^9/L E plaquetas ≤40 ×10^9/L — elegíveis para o efeitoRF-SANZ-LOW-RISK- APL com baixo risco para o Sanz — WBC ≤10 ×10^9/L E plaquetas >40 ×10^9/L — suporta ATRA+ATO ch...
Fontes
SRC-APL-RELAPSE-LENGFELDER-2017- Resultados de pacientes com recidiva de leucemia promielocítica aguda tratados com...SRC-APL0406-LOCOCO-2013- Ácido retinoico e trióxido de arsênico para leucemia promielocítica agudaSRC-ELN-AML-2022- Diagnosis and management of AML in adults: 2022 ELN recommendations from an international...SRC-ELN-APL-2019- Gestão da leucemia promielocítica aguda: recomendações atualizadas de um painel de peritos...SRC-ESMO-AML-2020- Acute myeloid leukaemia in adult patients: ESMO Clinical Practice Guidelines for diagnosi...SRC-MYLOFRANCE-ARIBI-2007- Terapia combinada com trióxido de arsênico para recidiva de leucemia promielocítica aguda