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Higher Risk Myelodysplastic Syndrome

MedGen UID:
1686380
•Concept ID:
C5207034
•
Finding
Synonym: Higher-Risk Myelodysplastic Syndrome

Definition

Myelodysplastic syndrome with a score of INT-2 (intermediate-2) or higher (the category above INT-2). [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHigher Risk Myelodysplastic Syndrome

Professional guidelines

PubMed

Current State of the Art: Management of Higher Risk Myelodysplastic Syndromes.
Komrokji RS
Clin Lymphoma Myeloma Leuk 2016 Aug;16 Suppl:S39-43. doi: 10.1016/j.clml.2016.02.019. PMID: 27521322
Treatment of advanced myelodysplastic syndrome with demethylating agents: azacitidine.
Adès L, Itzykson R, Fenaux P
Semin Hematol 2012 Oct;49(4):323-9. doi: 10.1053/j.seminhematol.2012.09.002. PMID: 23079062
Treatment of higher-risk myelodysplastic syndrome.
Garcia-Manero G
Semin Oncol 2011 Oct;38(5):673-81. doi: 10.1053/j.seminoncol.2011.07.001. PMID: 21943674

Recent clinical studies

Etiology

CPX-351 in higher risk myelodysplastic syndrome and chronic myelomonocytic leukaemia: a multicentre, single-arm, phase 2 study.
Peterlin P, Le Bris Y, Turlure P, Chevallier P, Ménard A, Gourin MP, Dumas PY, Thepot S, Berceanu A, Park S, Hospital MA, Cluzeau T, Bouzy S, Torregrosa-Diaz JM, Drevon L, Sapena R, Chermat F, Ades L, Dimicoli-Salazar S, Chevret S, Béné MC, Fenaux P
Lancet Haematol 2023 Jul;10(7):e521-e529. Epub 2023 May 25 doi: 10.1016/S2352-3026(23)00090-X. PMID: 37245522
The cure of leukemia through the optimist's prism.
Kantarjian HM, Jain N, Garcia-Manero G, Welch MA, Ravandi F, Wierda WG, Jabbour EJ
Cancer 2022 Jan 15;128(2):240-259. Epub 2021 Oct 6 doi: 10.1002/cncr.33933. PMID: 34614211Free PMC Article
Predicting outcome in higher-risk myelodysplastic syndrome patients treated with azacitidine.
Bouchla A, Thomopoulos TP, Papageorgiou SG, Apostolopoulou C, Loucari C, Mpazani E, Pappa V
Epigenomics 2021 Jul;13(14):1129-1143. Epub 2021 Jul 22 doi: 10.2217/epi-2021-0124. PMID: 34291653
Hypomethylating agent based combinations in higher risk myelodysplastic syndrome.
Chandhok NS, Lewis R, Prebet T
Leuk Lymphoma 2020 May;61(5):1012-1027. Epub 2019 Dec 9 doi: 10.1080/10428194.2019.1697812. PMID: 31814484
Stopping higher-risk myelodysplastic syndrome in its tracks.
Pollyea DA, Gutman JA
Curr Hematol Malig Rep 2014 Dec;9(4):421-31. doi: 10.1007/s11899-014-0234-1. PMID: 25208927

Diagnosis

CPX-351 in higher risk myelodysplastic syndrome and chronic myelomonocytic leukaemia: a multicentre, single-arm, phase 2 study.
Peterlin P, Le Bris Y, Turlure P, Chevallier P, Ménard A, Gourin MP, Dumas PY, Thepot S, Berceanu A, Park S, Hospital MA, Cluzeau T, Bouzy S, Torregrosa-Diaz JM, Drevon L, Sapena R, Chermat F, Ades L, Dimicoli-Salazar S, Chevret S, Béné MC, Fenaux P
Lancet Haematol 2023 Jul;10(7):e521-e529. Epub 2023 May 25 doi: 10.1016/S2352-3026(23)00090-X. PMID: 37245522
Predicting outcome in higher-risk myelodysplastic syndrome patients treated with azacitidine.
Bouchla A, Thomopoulos TP, Papageorgiou SG, Apostolopoulou C, Loucari C, Mpazani E, Pappa V
Epigenomics 2021 Jul;13(14):1129-1143. Epub 2021 Jul 22 doi: 10.2217/epi-2021-0124. PMID: 34291653
Current State of the Art: Management of Higher Risk Myelodysplastic Syndromes.
Komrokji RS
Clin Lymphoma Myeloma Leuk 2016 Aug;16 Suppl:S39-43. doi: 10.1016/j.clml.2016.02.019. PMID: 27521322
Managing patients with higher-risk myelodysplastic syndrome with stable disease on hypomethylating agents.
Sekeres MA
Leuk Lymphoma 2015;56(12):3267-9. Epub 2015 Sep 11 doi: 10.3109/10428194.2015.1063149. PMID: 26077360
Stopping higher-risk myelodysplastic syndrome in its tracks.
Pollyea DA, Gutman JA
Curr Hematol Malig Rep 2014 Dec;9(4):421-31. doi: 10.1007/s11899-014-0234-1. PMID: 25208927

Therapy

CPX-351 in higher risk myelodysplastic syndrome and chronic myelomonocytic leukaemia: a multicentre, single-arm, phase 2 study.
Peterlin P, Le Bris Y, Turlure P, Chevallier P, Ménard A, Gourin MP, Dumas PY, Thepot S, Berceanu A, Park S, Hospital MA, Cluzeau T, Bouzy S, Torregrosa-Diaz JM, Drevon L, Sapena R, Chermat F, Ades L, Dimicoli-Salazar S, Chevret S, Béné MC, Fenaux P
Lancet Haematol 2023 Jul;10(7):e521-e529. Epub 2023 May 25 doi: 10.1016/S2352-3026(23)00090-X. PMID: 37245522
Predicting outcome in higher-risk myelodysplastic syndrome patients treated with azacitidine.
Bouchla A, Thomopoulos TP, Papageorgiou SG, Apostolopoulou C, Loucari C, Mpazani E, Pappa V
Epigenomics 2021 Jul;13(14):1129-1143. Epub 2021 Jul 22 doi: 10.2217/epi-2021-0124. PMID: 34291653
Hypomethylating agent based combinations in higher risk myelodysplastic syndrome.
Chandhok NS, Lewis R, Prebet T
Leuk Lymphoma 2020 May;61(5):1012-1027. Epub 2019 Dec 9 doi: 10.1080/10428194.2019.1697812. PMID: 31814484
In MDS, is higher risk higher reward?
Sanz GF
Hematology Am Soc Hematol Educ Program 2019 Dec 6;2019(1):381-390. doi: 10.1182/hematology.2019000042. PMID: 31808894Free PMC Article
Current State of the Art: Management of Higher Risk Myelodysplastic Syndromes.
Komrokji RS
Clin Lymphoma Myeloma Leuk 2016 Aug;16 Suppl:S39-43. doi: 10.1016/j.clml.2016.02.019. PMID: 27521322

Prognosis

CPX-351 in higher risk myelodysplastic syndrome and chronic myelomonocytic leukaemia: a multicentre, single-arm, phase 2 study.
Peterlin P, Le Bris Y, Turlure P, Chevallier P, Ménard A, Gourin MP, Dumas PY, Thepot S, Berceanu A, Park S, Hospital MA, Cluzeau T, Bouzy S, Torregrosa-Diaz JM, Drevon L, Sapena R, Chermat F, Ades L, Dimicoli-Salazar S, Chevret S, Béné MC, Fenaux P
Lancet Haematol 2023 Jul;10(7):e521-e529. Epub 2023 May 25 doi: 10.1016/S2352-3026(23)00090-X. PMID: 37245522
The cure of leukemia through the optimist's prism.
Kantarjian HM, Jain N, Garcia-Manero G, Welch MA, Ravandi F, Wierda WG, Jabbour EJ
Cancer 2022 Jan 15;128(2):240-259. Epub 2021 Oct 6 doi: 10.1002/cncr.33933. PMID: 34614211Free PMC Article
Predicting outcome in higher-risk myelodysplastic syndrome patients treated with azacitidine.
Bouchla A, Thomopoulos TP, Papageorgiou SG, Apostolopoulou C, Loucari C, Mpazani E, Pappa V
Epigenomics 2021 Jul;13(14):1129-1143. Epub 2021 Jul 22 doi: 10.2217/epi-2021-0124. PMID: 34291653
In MDS, is higher risk higher reward?
Sanz GF
Hematology Am Soc Hematol Educ Program 2019 Dec 6;2019(1):381-390. doi: 10.1182/hematology.2019000042. PMID: 31808894Free PMC Article
Stopping higher-risk myelodysplastic syndrome in its tracks.
Pollyea DA, Gutman JA
Curr Hematol Malig Rep 2014 Dec;9(4):421-31. doi: 10.1007/s11899-014-0234-1. PMID: 25208927

Clinical prediction guides

Azacitidine Monotherapy in Patients With Treatment-Naïve Higher-risk Myelodysplastic Syndrome: A Systematic Literature Review and Meta-analysis.
Hasegawa K, Wei AH, Garcia-Manero G, Daver NG, Rajakumaraswamy N, Iqbal S, Chan RJ, Hu H, Tse P, Yan J, Zoratti MJ, Xie F, Sallman DA
Clin Lymphoma Myeloma Leuk 2023 Feb;23(2):127-137. Epub 2022 Nov 6 doi: 10.1016/j.clml.2022.11.002. PMID: 36428152
Predicting outcome in higher-risk myelodysplastic syndrome patients treated with azacitidine.
Bouchla A, Thomopoulos TP, Papageorgiou SG, Apostolopoulou C, Loucari C, Mpazani E, Pappa V
Epigenomics 2021 Jul;13(14):1129-1143. Epub 2021 Jul 22 doi: 10.2217/epi-2021-0124. PMID: 34291653
In MDS, is higher risk higher reward?
Sanz GF
Hematology Am Soc Hematol Educ Program 2019 Dec 6;2019(1):381-390. doi: 10.1182/hematology.2019000042. PMID: 31808894Free PMC Article
EVI1 expression predicts outcome in higher-risk myelodysplastic syndrome patients.
Liu L, Wang J, Jiang Y, Xie H, Tang X, Li Q, Wang H, Zou P, Miao Z, Lv Y, Wang H, Cao Z, Zhao Z
Leuk Lymphoma 2018 Dec;59(12):2929-2940. Epub 2018 May 30 doi: 10.1080/10428194.2018.1459615. PMID: 29846125
Current State of the Art: Management of Higher Risk Myelodysplastic Syndromes.
Komrokji RS
Clin Lymphoma Myeloma Leuk 2016 Aug;16 Suppl:S39-43. doi: 10.1016/j.clml.2016.02.019. PMID: 27521322

Recent systematic reviews

Azacitidine Monotherapy in Patients With Treatment-Naïve Higher-risk Myelodysplastic Syndrome: A Systematic Literature Review and Meta-analysis.
Hasegawa K, Wei AH, Garcia-Manero G, Daver NG, Rajakumaraswamy N, Iqbal S, Chan RJ, Hu H, Tse P, Yan J, Zoratti MJ, Xie F, Sallman DA
Clin Lymphoma Myeloma Leuk 2023 Feb;23(2):127-137. Epub 2022 Nov 6 doi: 10.1016/j.clml.2022.11.002. PMID: 36428152

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