Myelodysplastic Syndromes — Higher-Risk (IPSS-R high / very high; includes MDS-EB)
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-MDS-HR |
|---|---|
| Type | Disease |
| Aliases | HR-MDSHigher-risk MDSMDS with excess blastsMDS-EBMDS-HRMyelodysplastic Syndromes — Higher RiskRAEB (legacy FAB)Мієлодиспластичні синдроми, високий ризик (IPSS-R високий / дуже високий; включає MDS-EB) |
| Status | reviewed 2026-04-25 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-ESMO-MDS-2021 SRC-IPSS-M-BERNARD-2022 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 | 9983/3 |
|---|---|
| icd_o_3_topography | ["C42.1"] |
| icd_10 | D46.2 |
| who_classification | Myelodysplastic neoplasms with increased blasts (MDS-IB1, MDS-IB2 per WHO 5th ed.) — higher-risk subgroup |
Notes
Separated from MDS-LR because therapeutic intent (disease modification + transplant bridge) differs from cytopenia management. WHO 5th ed. reclassifies the older "MDS-EB" / "RAEB" terminology to MDS-IB1 (5-9% blasts) and MDS-IB2 (10-19% blasts). TP53-mutated MDS is its own WHO entity with poor HMA + alloHCT outcomes (active research question; no consensus best regimen). STUB — requires clinical co-lead signoff before publication. Myeloid expansion (heme).
Used By
Actionability
BMA-IDH1-R132H-MDS-HR- IDH1 R132 in MDS — ivosidenib monotherapy active (DiNardo et al. JCO 2021 — ORR 75% MDS,...BMA-IDH2-R140Q-MDS-HR- IDH2 R140Q in MDS — enasidenib monotherapy active in MDS (NCT01915498, DiNardo et al. Blo...BMA-IDH2-R172K-MDS-HR- IDH2 R172K in MDS — same enasidenib rationale as R140Q.BMA-NRAS-G12-MDS-HR- NRAS G12 in MDS-HR — adverse marker. Azacitidine + venetoclax / alloSCT.BMA-NRAS-G13-MDS-HR- NRAS G13 in MDS-HR — adverse marker. Azacitidine + venetoclax / alloSCT.BMA-NRAS-Q61R-MDS-HR- NRAS in higher-risk MDS — adverse marker; HMA + venetoclax considered, alloSCT preferred.BMA-TP53-MUT-MDS-HR- TP53-mut higher-risk MDS — dismal prognosis with HMA monotherapy (median OS ~6-10 mo). Ep...BMA-TP53-R175H-MDS-HR- TP53 hotspot R175H in higher-risk MDS — dismal prognosis. Biallelic vs monoallelic per IP...BMA-TP53-R248Q-MDS-HR- TP53 hotspot R248Q in higher-risk MDS — dismal prognosis. Biallelic vs monoallelic per IP...BMA-TP53-R273H-MDS-HR- TP53 hotspot R273H in higher-risk MDS — dismal prognosis. Biallelic vs monoallelic per IP...BMA-TP53-R282W-MDS-HR- TP53 hotspot R282W in higher-risk MDS — dismal prognosis. Biallelic vs monoallelic per IP...
Algorithms
ALGO-MDS-HR-1L- ALGO-MDS-HR-1LALGO-MDS-HR-2L- ALGO-MDS-HR-2L
Disease
DIS-AML- Acute Myeloid Leukemia (non-APL)DIS-MDS-LR- Myelodysplastic Syndromes — Lower-Risk (IPSS-R very low / low / intermediate)DIS-PMF- Primary Myelofibrosis (DIPSS-Plus stratified)
Indication
IND-ANKRD26-CARRIER-INTENSIFIED- IND-ANKRD26-CARRIER-INTENSIFIEDIND-ANKRD26-CARRIER-SURVEILLANCE- IND-ANKRD26-CARRIER-SURVEILLANCEIND-DDX41-CARRIER-INTENSIFIED- IND-DDX41-CARRIER-INTENSIFIEDIND-DDX41-CARRIER-SURVEILLANCE- IND-DDX41-CARRIER-SURVEILLANCEIND-MDS-HR-1L-AZA- IND-MDS-HR-1L-AZAIND-MDS-HR-1L-VEN-AZA- IND-MDS-HR-1L-VEN-AZAIND-MDS-HR-ALLOHCT- IND-MDS-HR-ALLOHCTIND-OCC-BENZENE-PREVENTION-INTENSIFIED-SURVEILLANCE- IND-OCC-BENZENE-PREVENTION-INTENSIFIED-SURVEILLANCE
Questionnaires
QUEST-MDS-HR-1L-STUB- Myelodysplastic Syndromes — Higher Risk — first line
Red flag
RF-ANKRD26-CONFIRMED-CARRIER- Confirmed germline pathogenic / likely-pathogenic variant in ANKRD26 (Thrombocytopenia 2,...RF-DDX41-CONFIRMED-CARRIER- Confirmed germline pathogenic / likely-pathogenic variant in DDX41 — most commonly the p....RF-ETV6-CONFIRMED-CARRIER- Confirmed germline pathogenic / likely-pathogenic variant in ETV6 (Thrombocytopenia 5, TH...RF-IATROGENIC-ASCT-SECONDARY-MDS-AML-PREVENTION- History of autologous stem-cell transplantation (ASCT) as consolidation or salvage therap...RF-IPSS-M-HIGH- MDS with IPSS-M high or very-high risk (2022 molecular update incorporating 31 gene mutat...RF-IPSS-R-VERY-HIGH- Higher-risk MDS with IPSS-R very-high category (score >6) — alloHCT primary curative path...RF-MDS-FRAILTY-AGE- MDS patient elderly or frail (age ≥80, ECOG ≥3, life expectancy <2 years, multiple comorb...RF-MDS-HIGH-RISK-IPSS- MDS escalates from lower-risk to higher-risk classification by IPSS-R (high / very high,...RF-MDS-INFECTION-SCREENING- MDS patient with positive HBV / HCV / HIV serology, latent TB, or active uncontrolled inf...RF-MDS-ORGAN-DYSFUNCTION- MDS patient with organ dysfunction limiting therapy: ECOG ≥3, severe renal impairment (Cr...RF-MDS-TP53-MUTATION- MDS with TP53 mutation (mono- or biallelic) — distinct WHO 5th-ed entity with poor outcom...RF-MDS-TRANSFORMATION-PROGRESSION- MDS progressing to AML (≥20% blasts) or accelerated MDS-IB2 with rapid progression on HMA...RF-MDS-TRANSPLANT-ELIGIBLE- MDS-HR patient transplant-eligible: age <70-75 (per local practice), HCT-CI ≤4, adequate...RF-OCC-BENZENE-MALIGNANCY-PREVENTION- Documented historical occupational exposure to benzene in an individual without a current...RF-RUNX1-CONFIRMED-CARRIER- Confirmed germline pathogenic / likely-pathogenic variant in RUNX1 (Familial Platelet Dis...
Sources
SRC-ESMO-MDS-2021- Myelodysplastic syndromes: ESMO Clinical Practice Guidelines for diagnosis, treatment and...SRC-IPSS-M-BERNARD-2022- Molecular International Prognostic Scoring System for Myelodysplastic Syndromes