Linfangioleiomiomatose (LAM)
Visão determinística da entidade fonte YAML. A autoridade clínica permanece com os IDs de fonte citados e estado de assinatura do revisor.
| ID | DIS-LAM |
|---|---|
| Tipo | Doença |
| Alegais | LAMLinfangioleiomiomatoseLAM pulmonarLAM esporádicaLAM associada à esclerose tuberosaЛімфангіолейоміоматоз (ЛАМ) |
| Estado | reviewed 2026-05-04 | pending_clinical_signoff |
| Doenças | Nenhuma declaração |
| Fontes | SRC-MILES-MCCORMACK-2011 SRC-NCCN-SARCOMA |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 8894/1 |
|---|---|
| icd_o_3_topography | ["C34"] |
| icd_10 | J84.81 |
| who_classification | WHO Classification of Thoracic Tumours 5th ed (2021): Lymphangioleiomyomatosis (PEComa family) |
Notas
LAM is a rare, progressive cystic lung disease caused by TSC1/TSC2 loss-of-function and consequent mTORC1 hyperactivation. Sirolimus (rapamycin) is FDA/EMA-approved for LAM (MILES trial, 2011: improved FEV1 trajectory, reduced VEGF-D). Everolimus is used for TSC-related manifestations including SEGA and renal AML. Disease is estrogen-sensitive: exacerbations during pregnancy, use of hormonal contraceptives discouraged. Diagnosis: high-resolution CT (diffuse bilateral thin-walled cysts) ± serum VEGF-D ≥800 pg/mL (diagnostic without biopsy in right clinical context). LAM Foundation guidelines (NHLBI/ERS) primary reference; NCCN does not cover LAM.
Usado por
Relevância terapêutica
BMA-TSC1-LAM- Sirolimus (rapamycin), um mTORC1 inibidor, é FDA-aprovado (Maio) 2015) e EMA-aprovado (...BMA-TSC1-TSC-RENAL-AML- Everolimus (mTOR inhibitor) is FDA-approved (2012) for renal angiomyolipoma (AML) associa...
Algoritmos
ALGO-LAM-1L- ALGO-LAM-1L
Indicação
IND-LAM-1L-SIROLIMUS- IND-LAM-1L-SIROLIMUS
Questionários
QUEST-LAM-1L-STUB- Linfangioleiomiomatose — primeira linha
Sinal de alerta
RF-TSC-CONFIRMED-CARRIER- Variância patogênica / patogênica provável confirmada da linha germinal em TSC1 (hamartin) ou TSC2 (tub...