Shimoyama smolding ou subtipo crônico-favorável de ATLL. Smoldering: ≥5% circulando...
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| ID | RF-ATLL-SHIMOYAMA-INDOLENT |
|---|---|
| Tipo | Sinal de alerta |
| Estado | revisto 2026-05-08 |
| Doenças | DIS-ATLL |
| Fontes | SRC-ESMO-PTCL-2024 SRC-NCCN-BCELL-2025 |
Origem da bandeira vermelha
| Definição | Shimoyama smoldering or chronic-favourable subtype of ATLL. Smoldering: ≥5% circulating abnormal lymphocytes, no organ involvement (skin/lung lesion permitted), LDH normal, BUN normal, albumin normal, no hypercalcemia, no lymphadenopathy or organomegaly. Chronic-favourable: similar to smoldering but lymphadenopathy/organomegaly permitted, LDH ≤2× ULN, BUN ≤ULN, albumin ≥ULN, Ca normal, no CNS/bone/GI/effusion. AZT+IFN-α (anti-HTLV-1 mechanism) yields 5-yr OS ~46% vs ~20% for chemotherapy in these subtypes (Bazarbachi 2010 meta-analysis, JNCI). Aggressive subtypes (acute/lymphoma/chronic-unfavourable) do NOT respond to AZT+IFN — route those to CHOEP+mogamulizumab via RF-ATLL-HIGH-RISK-BIOLOGY. |
|---|---|
| Direcção clínica | de escalonamento |
| Categoria | Outros |
| Algoritmo de deslocamentos | ALGO-ATLL-1L |
Lógica de Desencadeamento
{
"any_of": [
{
"finding": "shimoyama_subtype",
"value": "smoldering"
},
{
"finding": "shimoyama_subtype",
"value": "chronic_favourable"
},
{
"finding": "shimoyama_subtype",
"value": "chronic-favourable"
},
{
"finding": "atll_subtype",
"value": "smoldering"
},
{
"finding": "atll_subtype",
"value": "chronic_favourable"
},
{
"finding": "atll_subtype",
"value": "chronic-favourable"
},
{
"finding": "shimoyama_indolent",
"value": true
}
],
"type": "clinical_classification"
}
Notas
Shimoyama 1991 (JNCI 83:1034) is the gold-standard ATLL prognostic classifier. Four subtypes: smoldering / chronic-favourable / chronic- unfavourable / acute / lymphoma. This RF captures only the indolent end; chronic-unfavourable routes to aggressive track via lack of this RF (falls through to CHOEP default). Draft pending two-reviewer clinical co-lead signoff (CHARTER §6.1). Exclusion safety: step 1 screens for RF-ATLL-ORGAN-DYSFUNCTION + RF-ATLL-HIGH-RISK-BIOLOGY first — this RF only fires at step 2 after those are negative, so hypercalcemia/aggressive-subtype patients are already routed to CHOEP before this RF is evaluated.
Usado por
Algoritmos
ALGO-ATLL-1L- ALGO-ATLL-1L