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Myelodysplastic syndrome with excess blasts-2

MedGen UID:
962564
•Concept ID:
CN278404
•
Disease or Syndrome
Synonyms: MDS-EB-2; myelodysplastic syndrome with excess blasts-2; myelodysplastic syndrome with Excess blasts-2; RAEB-2; RAEB-II; refractory anaemia with excess blasts type 2; refractory anemia with excess blasts type 2; Refractory anemia with excess blasts type 2
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0015041
Orphanet: ORPHA100020

Definition

A very severe type of RAEB characterized by cytopenias and the following hematological parameters: uni- or multilineage dysplasia, 10% to 19% blasts in bone marrow or 5% to 19% in peripheral blood, variable presence of Auer rods (abnormal, needle-shaped or round inclusions in the cytoplasm of myeloblasts and promyelocytes). Median survival has been reported to be 18 months. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMyelodysplastic syndrome with excess blasts-2

Professional guidelines

PubMed

FLAGIDA-lite is an effective regimen for patients between 70 and 80 years with acute myeloid leukemia or refractory anemia with excess blasts-2 and is feasible as outpatient treatment.
Bermúdez A, Pérez-Vázquez G, Insunza A, Baro J, Colorado M, Conde E, Díez-Gallarreta Z, Gutiérrez ML, López-Duarte M, Olalla I, Sanroma P, Yañez L, Iriondo A, Richard C
Am J Hematol 2012 Jan;87(1):42-4. Epub 2011 Oct 20 doi: 10.1002/ajh.22192. PMID: 22015963
Leukemia incidence following primary breast carcinoma treatment.
Kaplan HG, Malmgren JA, Atwood M
Cancer 2004 Oct 1;101(7):1529-36. doi: 10.1002/cncr.20475. PMID: 15378478
13-cis-Retinoic acid treatment in patients with myelodysplastic syndrome.
Leoni F, Ciolli S, Longo G, Messori A, Ferrini PR
Acta Haematol 1988;80(1):8-12. doi: 10.1159/000205602. PMID: 3135696

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Myelodysplastic Syndromes, 2024

Recent clinical studies

Etiology

WT1 gene mutations impact post-transplant relapse in myelodysplastic syndrome with excess blasts 2 patients.
Guo W, Zhang H, Wang M, Zheng Y, Cao Y, Zhang X, Zhai W, Zhang R, Yang D, Wei J, He Y, Ma Q, Xia Y, Pang A, Feng S, Han M, Jiang E
Ann Hematol 2024 Aug;103(8):2827-2836. Epub 2024 Jul 6 doi: 10.1007/s00277-024-05870-1. PMID: 38969929
De Novo myelodysplastic syndromes in patients 20-50 years old are enriched for adverse risk features.
Epstein-Peterson ZD, Spitzer B, Derkach A, Arango JE, McCarter JGW, Medina-Martínez JS, McGovern E, Farnoud NR, Levine RL, Tallman MS
Leuk Res 2022 Jun;117:106857. Epub 2022 May 10 doi: 10.1016/j.leukres.2022.106857. PMID: 35598475Free PMC Article
Myeloid neoplasms associated with t(3;12)(q26.2;p13) are clinically aggressive, show myelodysplasia, and frequently harbor chromosome 7 abnormalities.
Ronaghy A, Hu S, Tang Z, Wang W, Tang G, Loghavi S, Li S, Thakral B, Medeiros LJ, Muzzafar T
Mod Pathol 2021 Feb;34(2):300-313. Epub 2020 Oct 27 doi: 10.1038/s41379-020-00663-z. PMID: 33110238
Epigenetic changes in FOXO3 and CHEK2 genes and their correlation with clinicopathological findings in myelodysplastic syndromes.
Sharifi MJ, Zaker F, Nasiri N, Yaghmaie M
Hematol Oncol Stem Cell Ther 2020 Dec;13(4):214-219. Epub 2020 Mar 18 doi: 10.1016/j.hemonc.2019.11.004. PMID: 32217071
Patients With a History of Chemotherapy and Isolated del(20q) With Minimal Myelodysplasia Have an Indolent Course.
Courville EL, Singh C, Yohe S, Linden MA, Naemi K, Berger M, Ustun C, McKenna RW, Dolan M
Am J Clin Pathol 2016 Apr;145(4):459-66. Epub 2016 Mar 25 doi: 10.1093/ajcp/aqw024. PMID: 27124938

Diagnosis

Acute Promyelocytic Leukemia Suggesting a Diagnosis of Myelodysplastic Syndrome: a Diagnostic Dilemma.
Shang H, Zhao L, Li R
Clin Lab 2022 Sep 1;68(9) doi: 10.7754/Clin.Lab.2021.211139. PMID: 36125158
De Novo myelodysplastic syndromes in patients 20-50 years old are enriched for adverse risk features.
Epstein-Peterson ZD, Spitzer B, Derkach A, Arango JE, McCarter JGW, Medina-Martínez JS, McGovern E, Farnoud NR, Levine RL, Tallman MS
Leuk Res 2022 Jun;117:106857. Epub 2022 May 10 doi: 10.1016/j.leukres.2022.106857. PMID: 35598475Free PMC Article
Myeloid neoplasms associated with t(3;12)(q26.2;p13) are clinically aggressive, show myelodysplasia, and frequently harbor chromosome 7 abnormalities.
Ronaghy A, Hu S, Tang Z, Wang W, Tang G, Loghavi S, Li S, Thakral B, Medeiros LJ, Muzzafar T
Mod Pathol 2021 Feb;34(2):300-313. Epub 2020 Oct 27 doi: 10.1038/s41379-020-00663-z. PMID: 33110238
Epigenetic changes in FOXO3 and CHEK2 genes and their correlation with clinicopathological findings in myelodysplastic syndromes.
Sharifi MJ, Zaker F, Nasiri N, Yaghmaie M
Hematol Oncol Stem Cell Ther 2020 Dec;13(4):214-219. Epub 2020 Mar 18 doi: 10.1016/j.hemonc.2019.11.004. PMID: 32217071
Patients With a History of Chemotherapy and Isolated del(20q) With Minimal Myelodysplasia Have an Indolent Course.
Courville EL, Singh C, Yohe S, Linden MA, Naemi K, Berger M, Ustun C, McKenna RW, Dolan M
Am J Clin Pathol 2016 Apr;145(4):459-66. Epub 2016 Mar 25 doi: 10.1093/ajcp/aqw024. PMID: 27124938

Therapy

Case Series Using Salvage Haplo-Identical Stem Cells for Secondary Transplantation.
Ionete A, Varady Z, Szegedi O, Coriu D
Medicina (Kaunas) 2023 Jun 2;59(6) doi: 10.3390/medicina59061077. PMID: 37374281Free PMC Article
Histone Deacetylase Inhibition with Panobinostat Combined with Intensive Induction Chemotherapy in Older Patients with Acute Myeloid Leukemia: Phase I Study Results.
Wieduwilt MJ, Pawlowska N, Thomas S, Olin R, Logan AC, Damon LE, Martin T, Kang M, Sayre PH, Boyer W, Gaensler KML, Anderson K, Munster PN, Andreadis C
Clin Cancer Res 2019 Aug 15;25(16):4917-4923. Epub 2019 May 31 doi: 10.1158/1078-0432.CCR-19-0171. PMID: 31152020
Patients With a History of Chemotherapy and Isolated del(20q) With Minimal Myelodysplasia Have an Indolent Course.
Courville EL, Singh C, Yohe S, Linden MA, Naemi K, Berger M, Ustun C, McKenna RW, Dolan M
Am J Clin Pathol 2016 Apr;145(4):459-66. Epub 2016 Mar 25 doi: 10.1093/ajcp/aqw024. PMID: 27124938
Myelodysplastic Syndrome Revealed by Systems Immunology in a Melanoma Patient Undergoing Anti-PD-1 Therapy.
Greenplate AR, Johnson DB, Roussel M, Savona MR, Sosman JA, Puzanov I, Ferrell PB, Irish JM
Cancer Immunol Res 2016 Jun;4(6):474-480. Epub 2016 Mar 10 doi: 10.1158/2326-6066.CIR-15-0213. PMID: 26966176Free PMC Article
A phase I trial of vorinostat and alvocidib in patients with relapsed, refractory, or poor prognosis acute leukemia, or refractory anemia with excess blasts-2.
Holkova B, Supko JG, Ames MM, Reid JM, Shapiro GI, Perkins EB, Ramakrishnan V, Tombes MB, Honeycutt C, McGovern RM, Kmieciak M, Shrader E, Wellons MD, Sankala H, Doyle A, Wright J, Roberts JD, Grant S
Clin Cancer Res 2013 Apr 1;19(7):1873-83. Epub 2013 Mar 20 doi: 10.1158/1078-0432.CCR-12-2926. PMID: 23515411Free PMC Article

Prognosis

De Novo myelodysplastic syndromes in patients 20-50 years old are enriched for adverse risk features.
Epstein-Peterson ZD, Spitzer B, Derkach A, Arango JE, McCarter JGW, Medina-Martínez JS, McGovern E, Farnoud NR, Levine RL, Tallman MS
Leuk Res 2022 Jun;117:106857. Epub 2022 May 10 doi: 10.1016/j.leukres.2022.106857. PMID: 35598475Free PMC Article
Myeloid neoplasms associated with t(3;12)(q26.2;p13) are clinically aggressive, show myelodysplasia, and frequently harbor chromosome 7 abnormalities.
Ronaghy A, Hu S, Tang Z, Wang W, Tang G, Loghavi S, Li S, Thakral B, Medeiros LJ, Muzzafar T
Mod Pathol 2021 Feb;34(2):300-313. Epub 2020 Oct 27 doi: 10.1038/s41379-020-00663-z. PMID: 33110238
Epigenetic changes in FOXO3 and CHEK2 genes and their correlation with clinicopathological findings in myelodysplastic syndromes.
Sharifi MJ, Zaker F, Nasiri N, Yaghmaie M
Hematol Oncol Stem Cell Ther 2020 Dec;13(4):214-219. Epub 2020 Mar 18 doi: 10.1016/j.hemonc.2019.11.004. PMID: 32217071
Patients With a History of Chemotherapy and Isolated del(20q) With Minimal Myelodysplasia Have an Indolent Course.
Courville EL, Singh C, Yohe S, Linden MA, Naemi K, Berger M, Ustun C, McKenna RW, Dolan M
Am J Clin Pathol 2016 Apr;145(4):459-66. Epub 2016 Mar 25 doi: 10.1093/ajcp/aqw024. PMID: 27124938
Cytogenetic characteristics and prognosis analysis in 231 myelodysplastic syndrome patients from a single institution.
Jung SW, Lee SY, Jekarl DW, Kim M, Lim J, Kim Y, Han K, Kim YJ, Cho SG, Song J
Leuk Res 2011 Jun;35(6):735-40. Epub 2010 Dec 13 doi: 10.1016/j.leukres.2010.11.009. PMID: 21146871

Clinical prediction guides

De Novo myelodysplastic syndromes in patients 20-50 years old are enriched for adverse risk features.
Epstein-Peterson ZD, Spitzer B, Derkach A, Arango JE, McCarter JGW, Medina-Martínez JS, McGovern E, Farnoud NR, Levine RL, Tallman MS
Leuk Res 2022 Jun;117:106857. Epub 2022 May 10 doi: 10.1016/j.leukres.2022.106857. PMID: 35598475Free PMC Article
Spectrum of Myelodysplastic Syndrome in Patients Evaluated for Cytopenia(s). A Report from a Reference Centre in Saudi Arabia.
AlMozain N, Mashi A, Alneami Q, Al-Omran A, Bakshi N, Owaidah T, Khalil S, Khogeer H, Hashmi S, Al-Sweedan S, Morris T, AlNounou R
Hematol Oncol Stem Cell Ther 2022 Jun 1;15(2):39-44. doi: 10.1016/j.hemonc.2020.11.001. PMID: 33227261
Epigenetic changes in FOXO3 and CHEK2 genes and their correlation with clinicopathological findings in myelodysplastic syndromes.
Sharifi MJ, Zaker F, Nasiri N, Yaghmaie M
Hematol Oncol Stem Cell Ther 2020 Dec;13(4):214-219. Epub 2020 Mar 18 doi: 10.1016/j.hemonc.2019.11.004. PMID: 32217071
Histone Deacetylase Inhibition with Panobinostat Combined with Intensive Induction Chemotherapy in Older Patients with Acute Myeloid Leukemia: Phase I Study Results.
Wieduwilt MJ, Pawlowska N, Thomas S, Olin R, Logan AC, Damon LE, Martin T, Kang M, Sayre PH, Boyer W, Gaensler KML, Anderson K, Munster PN, Andreadis C
Clin Cancer Res 2019 Aug 15;25(16):4917-4923. Epub 2019 May 31 doi: 10.1158/1078-0432.CCR-19-0171. PMID: 31152020
Cytogenetic characteristics and prognosis analysis in 231 myelodysplastic syndrome patients from a single institution.
Jung SW, Lee SY, Jekarl DW, Kim M, Lim J, Kim Y, Han K, Kim YJ, Cho SG, Song J
Leuk Res 2011 Jun;35(6):735-40. Epub 2010 Dec 13 doi: 10.1016/j.leukres.2010.11.009. PMID: 21146871

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    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Myelodysplastic Syndromes, 2024

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