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Very High Risk Myelodysplastic Syndrome

MedGen UID:
1720312
•Concept ID:
C5237066
•
Neoplastic Process

Definition

Myelodysplastic syndrome with more than 6 points, according to the revised International Prognostic Scoring System (IPSS-R). [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVery High Risk Myelodysplastic Syndrome

Professional guidelines

PubMed

New Approaches to Myelodysplastic Syndrome Treatment.
Bazinet A, Bravo GM
Curr Treat Options Oncol 2022 May;23(5):668-687. Epub 2022 Mar 23 doi: 10.1007/s11864-022-00965-1. PMID: 35320468
Chronic myelomonocytic leukemia: 2022 update on diagnosis, risk stratification, and management.
Patnaik MM, Tefferi A
Am J Hematol 2022 Mar 1;97(3):352-372. Epub 2022 Jan 27 doi: 10.1002/ajh.26455. PMID: 34985762
Diagnosis and management of Aspergillus diseases: executive summary of the 2017 ESCMID-ECMM-ERS guideline.
Ullmann AJ, Aguado JM, Arikan-Akdagli S, Denning DW, Groll AH, Lagrou K, Lass-Flörl C, Lewis RE, Munoz P, Verweij PE, Warris A, Ader F, Akova M, Arendrup MC, Barnes RA, Beigelman-Aubry C, Blot S, Bouza E, Brüggemann RJM, Buchheidt D, Cadranel J, Castagnola E, Chakrabarti A, Cuenca-Estrella M, Dimopoulos G, Fortun J, Gangneux JP, Garbino J, Heinz WJ, Herbrecht R, Heussel CP, Kibbler CC, Klimko N, Kullberg BJ, Lange C, Lehrnbecher T, Löffler J, Lortholary O, Maertens J, Marchetti O, Meis JF, Pagano L, Ribaud P, Richardson M, Roilides E, Ruhnke M, Sanguinetti M, Sheppard DC, Sinkó J, Skiada A, Vehreschild MJGT, Viscoli C, Cornely OA
Clin Microbiol Infect 2018 May;24 Suppl 1:e1-e38. Epub 2018 Mar 12 doi: 10.1016/j.cmi.2018.01.002. PMID: 29544767

Recent clinical studies

Etiology

Allogeneic Hematopoietic Cell Transplantation Improves Outcome in Myelodysplastic Syndrome Across High-Risk Genetic Subgroups: Genetic Analysis of the Blood and Marrow Transplant Clinical Trials Network 1102 Study.
Versluis J, Saber W, Tsai HK, Gibson CJ, Dillon LW, Mishra A, McGuirk J, Maziarz RT, Westervelt P, Hegde P, Mukherjee D, Martens MJ, Logan B, Horowitz M, Hourigan CS, Nakamura R, Cutler C, Lindsley RC; Blood and Marrow Transplant Clinical Trials Network
J Clin Oncol 2023 Oct 1;41(28):4497-4510. Epub 2023 Aug 22 doi: 10.1200/JCO.23.00866. PMID: 37607457Free PMC Article
Hematopoietic Cell Transplantation in the Management of Myelodysplastic Syndrome: An Evidence-Based Review from the American Society for Transplantation and Cellular Therapy Committee on Practice Guidelines.
DeFilipp Z, Ciurea SO, Cutler C, Robin M, Warlick ED, Nakamura R, Brunner AM, Dholaria B, Walker AR, Kröger N, Bejanyan N, Atallah E, Tamari R, Solh MM, Percival ME, de Lima M, Scott B, Oran B, Garcia-Manero G, Hamadani M, Carpenter P, DeZern AE
Transplant Cell Ther 2023 Feb;29(2):71-81. Epub 2022 Nov 25 doi: 10.1016/j.jtct.2022.11.014. PMID: 36436780
New Approaches to Myelodysplastic Syndrome Treatment.
Bazinet A, Bravo GM
Curr Treat Options Oncol 2022 May;23(5):668-687. Epub 2022 Mar 23 doi: 10.1007/s11864-022-00965-1. PMID: 35320468
Myelodysplastic syndrome.
Hofmann WK, Koeffler HP
Annu Rev Med 2005;56:1-16. doi: 10.1146/annurev.med.56.082103.104704. PMID: 15660498
International scoring system for evaluating prognosis in myelodysplastic syndromes.
Greenberg P, Cox C, LeBeau MM, Fenaux P, Morel P, Sanz G, Sanz M, Vallespi T, Hamblin T, Oscier D, Ohyashiki K, Toyama K, Aul C, Mufti G, Bennett J
Blood 1997 Mar 15;89(6):2079-88. PMID: 9058730

Diagnosis

Myelodysplastic Syndromes: New Methods of Diagnosis, Prognostication, and Treatment.
Nachtkamp K, Kobbe G, Gattermann N, Germing U
Dtsch Arztebl Int 2023 Mar 24;120(12):203-210. doi: 10.3238/arztebl.m2023.0005. PMID: 36718105Free PMC Article
New Approaches to Myelodysplastic Syndrome Treatment.
Bazinet A, Bravo GM
Curr Treat Options Oncol 2022 May;23(5):668-687. Epub 2022 Mar 23 doi: 10.1007/s11864-022-00965-1. PMID: 35320468
NCCN Guidelines® Insights: Myelodysplastic Syndromes, Version 3.2022.
Greenberg PL, Stone RM, Al-Kali A, Bennett JM, Borate U, Brunner AM, Chai-Ho W, Curtin P, de Castro CM, Deeg HJ, DeZern AE, Dinner S, Foucar C, Gaensler K, Garcia-Manero G, Griffiths EA, Head D, Jonas BA, Keel S, Madanat Y, Maness LJ, Mangan J, McCurdy S, McMahon C, Patel B, Reddy VV, Sallman DA, Shallis R, Shami PJ, Thota S, Varshavsky-Yanovsky AN, Westervelt P, Hollinger E, Shead DA, Hochstetler C
J Natl Compr Canc Netw 2022 Feb;20(2):106-117. doi: 10.6004/jnccn.2022.0009. PMID: 35130502
Molecular characterization of mutant TP53 acute myeloid leukemia and high-risk myelodysplastic syndrome.
Grob T, Al Hinai ASA, Sanders MA, Kavelaars FG, Rijken M, Gradowska PL, Biemond BJ, Breems DA, Maertens J, van Marwijk Kooy M, Pabst T, de Weerdt O, Ossenkoppele GJ, van de Loosdrecht AA, Huls GA, Cornelissen JJ, Beverloo HB, Löwenberg B, Jongen-Lavrencic M, Valk PJM
Blood 2022 Apr 14;139(15):2347-2354. doi: 10.1182/blood.2021014472. PMID: 35108372Free PMC Article
Venetoclax plus intensive chemotherapy with cladribine, idarubicin, and cytarabine in patients with newly diagnosed acute myeloid leukaemia or high-risk myelodysplastic syndrome: a cohort from a single-centre, single-arm, phase 2 trial.
Kadia TM, Reville PK, Borthakur G, Yilmaz M, Kornblau S, Alvarado Y, Dinardo CD, Daver N, Jain N, Pemmaraju N, Short N, Wang SA, Tidwell RSS, Islam R, Konopleva M, Garcia-Manero G, Ravandi F, Kantarjian HM
Lancet Haematol 2021 Aug;8(8):e552-e561. doi: 10.1016/S2352-3026(21)00192-7. PMID: 34329576Free PMC Article

Therapy

New Approaches to Myelodysplastic Syndrome Treatment.
Bazinet A, Bravo GM
Curr Treat Options Oncol 2022 May;23(5):668-687. Epub 2022 Mar 23 doi: 10.1007/s11864-022-00965-1. PMID: 35320468
Venetoclax plus intensive chemotherapy with cladribine, idarubicin, and cytarabine in patients with newly diagnosed acute myeloid leukaemia or high-risk myelodysplastic syndrome: a cohort from a single-centre, single-arm, phase 2 trial.
Kadia TM, Reville PK, Borthakur G, Yilmaz M, Kornblau S, Alvarado Y, Dinardo CD, Daver N, Jain N, Pemmaraju N, Short N, Wang SA, Tidwell RSS, Islam R, Konopleva M, Garcia-Manero G, Ravandi F, Kantarjian HM
Lancet Haematol 2021 Aug;8(8):e552-e561. doi: 10.1016/S2352-3026(21)00192-7. PMID: 34329576Free PMC Article
Secondary myelodysplastic syndrome and leukemia in acquired aplastic anemia and paroxysmal nocturnal hemoglobinuria.
Sun L, Babushok DV
Blood 2020 Jul 2;136(1):36-49. doi: 10.1182/blood.2019000940. PMID: 32430502Free PMC Article
Primary prophylaxis of invasive fungal infections in patients with haematological malignancies: 2017 update of the recommendations of the Infectious Diseases Working Party (AGIHO) of the German Society for Haematology and Medical Oncology (DGHO).
Mellinghoff SC, Panse J, Alakel N, Behre G, Buchheidt D, Christopeit M, Hasenkamp J, Kiehl M, Koldehoff M, Krause SW, Lehners N, von Lilienfeld-Toal M, Löhnert AY, Maschmeyer G, Teschner D, Ullmann AJ, Penack O, Ruhnke M, Mayer K, Ostermann H, Wolf HH, Cornely OA
Ann Hematol 2018 Feb;97(2):197-207. Epub 2017 Dec 7 doi: 10.1007/s00277-017-3196-2. PMID: 29218389Free PMC Article
Posaconazole vs. fluconazole or itraconazole prophylaxis in patients with neutropenia.
Cornely OA, Maertens J, Winston DJ, Perfect J, Ullmann AJ, Walsh TJ, Helfgott D, Holowiecki J, Stockelberg D, Goh YT, Petrini M, Hardalo C, Suresh R, Angulo-Gonzalez D
N Engl J Med 2007 Jan 25;356(4):348-59. doi: 10.1056/NEJMoa061094. PMID: 17251531

Prognosis

Allogeneic Hematopoietic Cell Transplantation Improves Outcome in Myelodysplastic Syndrome Across High-Risk Genetic Subgroups: Genetic Analysis of the Blood and Marrow Transplant Clinical Trials Network 1102 Study.
Versluis J, Saber W, Tsai HK, Gibson CJ, Dillon LW, Mishra A, McGuirk J, Maziarz RT, Westervelt P, Hegde P, Mukherjee D, Martens MJ, Logan B, Horowitz M, Hourigan CS, Nakamura R, Cutler C, Lindsley RC; Blood and Marrow Transplant Clinical Trials Network
J Clin Oncol 2023 Oct 1;41(28):4497-4510. Epub 2023 Aug 22 doi: 10.1200/JCO.23.00866. PMID: 37607457Free PMC Article
New Approaches to Myelodysplastic Syndrome Treatment.
Bazinet A, Bravo GM
Curr Treat Options Oncol 2022 May;23(5):668-687. Epub 2022 Mar 23 doi: 10.1007/s11864-022-00965-1. PMID: 35320468
Venetoclax plus intensive chemotherapy with cladribine, idarubicin, and cytarabine in patients with newly diagnosed acute myeloid leukaemia or high-risk myelodysplastic syndrome: a cohort from a single-centre, single-arm, phase 2 trial.
Kadia TM, Reville PK, Borthakur G, Yilmaz M, Kornblau S, Alvarado Y, Dinardo CD, Daver N, Jain N, Pemmaraju N, Short N, Wang SA, Tidwell RSS, Islam R, Konopleva M, Garcia-Manero G, Ravandi F, Kantarjian HM
Lancet Haematol 2021 Aug;8(8):e552-e561. doi: 10.1016/S2352-3026(21)00192-7. PMID: 34329576Free PMC Article
Myelodysplastic syndrome.
Hofmann WK, Koeffler HP
Annu Rev Med 2005;56:1-16. doi: 10.1146/annurev.med.56.082103.104704. PMID: 15660498
International scoring system for evaluating prognosis in myelodysplastic syndromes.
Greenberg P, Cox C, LeBeau MM, Fenaux P, Morel P, Sanz G, Sanz M, Vallespi T, Hamblin T, Oscier D, Ohyashiki K, Toyama K, Aul C, Mufti G, Bennett J
Blood 1997 Mar 15;89(6):2079-88. PMID: 9058730

Clinical prediction guides

Allogeneic Hematopoietic Cell Transplantation Improves Outcome in Myelodysplastic Syndrome Across High-Risk Genetic Subgroups: Genetic Analysis of the Blood and Marrow Transplant Clinical Trials Network 1102 Study.
Versluis J, Saber W, Tsai HK, Gibson CJ, Dillon LW, Mishra A, McGuirk J, Maziarz RT, Westervelt P, Hegde P, Mukherjee D, Martens MJ, Logan B, Horowitz M, Hourigan CS, Nakamura R, Cutler C, Lindsley RC; Blood and Marrow Transplant Clinical Trials Network
J Clin Oncol 2023 Oct 1;41(28):4497-4510. Epub 2023 Aug 22 doi: 10.1200/JCO.23.00866. PMID: 37607457Free PMC Article
Real-World Validation of Molecular International Prognostic Scoring System for Myelodysplastic Syndromes.
Sauta E, Robin M, Bersanelli M, Travaglino E, Meggendorfer M, Zhao LP, Caballero Berrocal JC, Sala C, Maggioni G, Bernardi M, Di Grazia C, Vago L, Rivoli G, Borin L, D'Amico S, Tentori CA, Ubezio M, Campagna A, Russo A, Mannina D, Lanino L, Chiusolo P, Giaccone L, Voso MT, Riva M, Oliva EN, Zampini M, Riva E, Nibourel O, Bicchieri M, Bolli N, Rambaldi A, Passamonti F, Savevski V, Santoro A, Germing U, Kordasti S, Santini V, Diez-Campelo M, Sanz G, Sole F, Kern W, Platzbecker U, Ades L, Fenaux P, Haferlach T, Castellani G, Della Porta MG
J Clin Oncol 2023 May 20;41(15):2827-2842. Epub 2023 Mar 17 doi: 10.1200/JCO.22.01784. PMID: 36930857Free PMC Article
New Approaches to Myelodysplastic Syndrome Treatment.
Bazinet A, Bravo GM
Curr Treat Options Oncol 2022 May;23(5):668-687. Epub 2022 Mar 23 doi: 10.1007/s11864-022-00965-1. PMID: 35320468
Molecular characterization of mutant TP53 acute myeloid leukemia and high-risk myelodysplastic syndrome.
Grob T, Al Hinai ASA, Sanders MA, Kavelaars FG, Rijken M, Gradowska PL, Biemond BJ, Breems DA, Maertens J, van Marwijk Kooy M, Pabst T, de Weerdt O, Ossenkoppele GJ, van de Loosdrecht AA, Huls GA, Cornelissen JJ, Beverloo HB, Löwenberg B, Jongen-Lavrencic M, Valk PJM
Blood 2022 Apr 14;139(15):2347-2354. doi: 10.1182/blood.2021014472. PMID: 35108372Free PMC Article
Venetoclax plus intensive chemotherapy with cladribine, idarubicin, and cytarabine in patients with newly diagnosed acute myeloid leukaemia or high-risk myelodysplastic syndrome: a cohort from a single-centre, single-arm, phase 2 trial.
Kadia TM, Reville PK, Borthakur G, Yilmaz M, Kornblau S, Alvarado Y, Dinardo CD, Daver N, Jain N, Pemmaraju N, Short N, Wang SA, Tidwell RSS, Islam R, Konopleva M, Garcia-Manero G, Ravandi F, Kantarjian HM
Lancet Haematol 2021 Aug;8(8):e552-e561. doi: 10.1016/S2352-3026(21)00192-7. PMID: 34329576Free PMC Article

Recent systematic reviews

Do anemia treatments improve quality of life and physical function in patients with myelodysplastic syndromes (MDS)? A systematic review.
Mo A, Poynton M, Wood E, Shortt J, Brunskill SJ, Doree C, Sandercock J, Saadah N, Luk E, Stanworth SJ, McQuilten Z
Blood Rev 2023 Sep;61:101114. Epub 2023 Jul 13 doi: 10.1016/j.blre.2023.101114. PMID: 37479599
Incidence and risk of hematologic toxicities with hypomethylating agents in the treatment of myelodysplastic syndromes and acute myeloid leukopenia: A systematic review and meta-analysis.
Gao C, Wang J, Li Y, Zhao H, Li R, Hou L, Zhang Y, Tian S, Liang H, Wang C, Chen X, Wang J
Medicine (Baltimore) 2018 Aug;97(34):e11860. doi: 10.1097/MD.0000000000011860. PMID: 30142779Free PMC Article
Overall survival and risk of second malignancies with cancer chemotherapy and G-CSF support.
Lyman GH, Yau L, Nakov R, Krendyukov A
Ann Oncol 2018 Sep 1;29(9):1903-1910. doi: 10.1093/annonc/mdy311. PMID: 30099478
Recommendations for the Use of WBC Growth Factors: American Society of Clinical Oncology Clinical Practice Guideline Update.
Smith TJ, Bohlke K, Lyman GH, Carson KR, Crawford J, Cross SJ, Goldberg JM, Khatcheressian JL, Leighl NB, Perkins CL, Somlo G, Wade JL, Wozniak AJ, Armitage JO; American Society of Clinical Oncology
J Clin Oncol 2015 Oct 1;33(28):3199-212. Epub 2015 Jul 13 doi: 10.1200/JCO.2015.62.3488. PMID: 26169616
Secondary malignancies following high dose therapy and autologous hematopoietic cell transplantation-systematic review and meta-analysis.
Vaxman I, Ram R, Gafter-Gvili A, Vidal L, Yeshurun M, Lahav M, Shpilberg O
Bone Marrow Transplant 2015 May;50(5):706-14. Epub 2015 Feb 9 doi: 10.1038/bmt.2014.325. PMID: 25665042

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