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TP53-mut higher-risk MDS — dismal prognosis with HMA monotherapy (median OS ~6-10 mo). Ep...

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

IDBMA-TP53-MUT-MDS-HR
TypeActionability
Statusreviewed 2026-04-27 | pending_clinical_signoff | actionability review required
DiseasesDIS-MDS-HR
SourcesSRC-CIVIC SRC-ESMO-MDS-2021 SRC-IPSS-M-BERNARD-2022

Actionability Facts

BiomarkerBIO-TP53-MUTATION
Variantany pathogenic mutation OR del(17p)
DiseaseDIS-MDS-HR
ESCAT tierIIIB
Recommended combinationsclinical trial enrollment, alloSCT consideration (preferred curative path despite poor outcomes), azacitidine + venetoclax (palliative; modest CR but non-durable)
Evidence summaryTP53-mut higher-risk MDS — dismal prognosis with HMA monotherapy (median OS ~6-10 mo). Eprenetapopt (APR-246) Ph3 missed primary endpoint and was retired (2021). AlloSCT remains the only potentially curative option but outcomes inferior to TP53-WT. Magrolimab + azacitidine Ph3 (ENHANCE) negative.

Notes

ESCAT IIIB. Gene-level cell — biallelic vs monoallelic distinction matters in MDS/AML (biallelic = TP53-multihit per IPSS-M / ICC 2022, far worse). Per-hotspot cells provided for the most common variants.

Used By

No reverse references found in the YAML corpus.