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Myelodysplastic syndrome with excess blasts

MedGen UID:
8066
Concept ID:
C0002894
Neoplastic Process
Synonyms: Anemia, Refractory, with Excess of Blasts; Leukemia, Smoldering; Leukemia, Smouldering; Leukemias, Smoldering; RAEB; RAEM; Refractory Anemia with Excess of Blasts; Smoldering Leukemia; Smoldering Leukemias; Smouldering Leukemia
SNOMED CT: Refractory anemia with excess blasts (398623004); RAEB - Refractory anemia with excess blasts (398623004); Refractory anemia with excess blasts (128847002); Myelodysplastic syndrome with excess blasts (128847002); Myelodysplastic syndrome with excess blasts (398623004); Myelodysplastic syndrome with increased blasts (128847002)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019454
Orphanet: ORPHA86839

Definition

Refractory anemia with excess blasts (RAEB) is a frequent severe subtype of myelodysplastic syndrome (MDS; see this term) characterized by cytopenias with unilineage or multilineage dysplasia and 5% to 19% blasts in bone marrow or blood. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMyelodysplastic syndrome with excess blasts
Follow this link to review classifications for Myelodysplastic syndrome with excess blasts in Orphanet.

Professional guidelines

PubMed

Lenaerts L, Vandenberghe P, Brison N, Che H, Neofytou M, Verheecke M, Leemans L, Maggen C, Dewaele B, Dehaspe L, Vanderschueren S, Dierickx D, Vandecaveye V, Amant F, Vermeesch JR
Ann Oncol 2019 Jan 1;30(1):85-95. doi: 10.1093/annonc/mdy476. PMID: 30371735

Recent clinical studies

Etiology

Nakagawa N, Ishiyama K, Usuki K, Takada S, Tomikawa T, Handa H, Katsuoka Y, Hirano D, Sezaki N, Sumi M, Fujisawa S, Taniguchi Y, Mugitani A, Yoshimura T, Ohtsuka E, Takase K, Suehiro Y, Ota S, Kajiguchi T, Maeda T, Yamamoto M, Ohtake S, Katsumi A, Kiyoi H, Matsumura I, Miyazaki Y
Ann Hematol 2024 Jan;103(1):307-320. Epub 2023 Nov 8 doi: 10.1007/s00277-023-05527-5. PMID: 37940714
Ma YY, Wei ZL, Xu YJ, Shi JM, Yi H, Lai YR, Jiang EL, Wang SB, Wu T, Gao L, Gao L, Kong PY, Wen Q, Bai H, Li Y, Cao YG, Li QC, Zhang ZM, Liu BC, Su Y, Lai XY, Ma X, Cheng TT, Luo Y, Zhang X, Zhang C
Cancer 2023 Jul 1;129(13):2013-2022. Epub 2023 Mar 23 doi: 10.1002/cncr.34762. PMID: 36951498
Shen K, Hu DY, Zhang ZB, Guo YS, Zhang FH, Chen SN
Int J Lab Hematol 2023 Jun;45(3):344-352. Epub 2023 Mar 1 doi: 10.1111/ijlh.14030. PMID: 36860196
Bernués M, González T, Corchete LA, Santos S, Durán MA, López-Andrade B, Riso LL, Martínez-Serra J, Ramos R, Iglesias J, Royo I, Rosell J
Cancer Genet 2022 Jun;264-265:60-65. Epub 2022 Mar 26 doi: 10.1016/j.cancergen.2022.03.004. PMID: 35397254
Chen X, Othus M, Wood BL, Walter RB, Becker PS, Percival ME, Abkowitz JL, Appelbaum FR, Estey EH
Leuk Lymphoma 2021 May;62(5):1226-1233. Epub 2020 Dec 19 doi: 10.1080/10428194.2020.1861267. PMID: 33345655

Diagnosis

Lee C, Kim HN, Kwon JA, Yoon SY, Jeon MJ, Yu ES, Kim DS, Choi CW, Yoon J
Ann Lab Med 2023 Sep 1;43(5):503-507. Epub 2023 Apr 21 doi: 10.3343/alm.2023.43.5.503. PMID: 37080752Free PMC Article
Ma YY, Wei ZL, Xu YJ, Shi JM, Yi H, Lai YR, Jiang EL, Wang SB, Wu T, Gao L, Gao L, Kong PY, Wen Q, Bai H, Li Y, Cao YG, Li QC, Zhang ZM, Liu BC, Su Y, Lai XY, Ma X, Cheng TT, Luo Y, Zhang X, Zhang C
Cancer 2023 Jul 1;129(13):2013-2022. Epub 2023 Mar 23 doi: 10.1002/cncr.34762. PMID: 36951498
Shen K, Hu DY, Zhang ZB, Guo YS, Zhang FH, Chen SN
Int J Lab Hematol 2023 Jun;45(3):344-352. Epub 2023 Mar 1 doi: 10.1111/ijlh.14030. PMID: 36860196
Shang H, Zhao L, Li R
Clin Lab 2022 Sep 1;68(9) doi: 10.7754/Clin.Lab.2021.211139. PMID: 36125158
Yan W, Qu XY, Li H, Li YC, Li Y, Yao K, Yang W, Wang HH
Ann Palliat Med 2021 Dec;10(12):12431-12440. doi: 10.21037/apm-21-3210. PMID: 35016480

Therapy

Nakagawa N, Ishiyama K, Usuki K, Takada S, Tomikawa T, Handa H, Katsuoka Y, Hirano D, Sezaki N, Sumi M, Fujisawa S, Taniguchi Y, Mugitani A, Yoshimura T, Ohtsuka E, Takase K, Suehiro Y, Ota S, Kajiguchi T, Maeda T, Yamamoto M, Ohtake S, Katsumi A, Kiyoi H, Matsumura I, Miyazaki Y
Ann Hematol 2024 Jan;103(1):307-320. Epub 2023 Nov 8 doi: 10.1007/s00277-023-05527-5. PMID: 37940714
Ho VT, Kim HT, Brock J, Galinsky I, Daley H, Reynolds C, Weber A, Pozdnyakova O, Severgnini M, Nikiforow S, Cutler C, Koreth J, Alyea EP, Antin JH, Gooptu M, Romee R, Shapiro R, Chen YB, Rosenblatt J, Avigan D, Hodi FS, Dranoff G, Wu CJ, Ritz J, Soiffer RJ
Blood Adv 2022 Apr 12;6(7):2183-2194. doi: 10.1182/bloodadvances.2021006255. PMID: 34807983Free PMC Article
Park HS, Son BR, Shin KS, Byeon S, Kim HK, Yang Y, Jeong Y, Han HS, Lee KH, Kwon J
Acta Haematol 2021;144(6):649-659. Epub 2021 Jul 7 doi: 10.1159/000516793. PMID: 34233332
Zhang C, Yao H, Kong PY, Liu Y, Gao L, Gao L, Ma YY, Liu J, Tan X, Zhang X
Anticancer Drugs 2019 Oct;30(9):948-952. doi: 10.1097/CAD.0000000000000811. PMID: 31283544Free PMC Article
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

Prognosis

Lee C, Kim HN, Kwon JA, Yoon SY, Jeon MJ, Yu ES, Kim DS, Choi CW, Yoon J
Ann Lab Med 2023 Sep 1;43(5):503-507. Epub 2023 Apr 21 doi: 10.3343/alm.2023.43.5.503. PMID: 37080752Free PMC Article
Ma YY, Wei ZL, Xu YJ, Shi JM, Yi H, Lai YR, Jiang EL, Wang SB, Wu T, Gao L, Gao L, Kong PY, Wen Q, Bai H, Li Y, Cao YG, Li QC, Zhang ZM, Liu BC, Su Y, Lai XY, Ma X, Cheng TT, Luo Y, Zhang X, Zhang C
Cancer 2023 Jul 1;129(13):2013-2022. Epub 2023 Mar 23 doi: 10.1002/cncr.34762. PMID: 36951498
Shen K, Hu DY, Zhang ZB, Guo YS, Zhang FH, Chen SN
Int J Lab Hematol 2023 Jun;45(3):344-352. Epub 2023 Mar 1 doi: 10.1111/ijlh.14030. PMID: 36860196
Bernués M, González T, Corchete LA, Santos S, Durán MA, López-Andrade B, Riso LL, Martínez-Serra J, Ramos R, Iglesias J, Royo I, Rosell J
Cancer Genet 2022 Jun;264-265:60-65. Epub 2022 Mar 26 doi: 10.1016/j.cancergen.2022.03.004. PMID: 35397254
Yan W, Qu XY, Li H, Li YC, Li Y, Yao K, Yang W, Wang HH
Ann Palliat Med 2021 Dec;10(12):12431-12440. doi: 10.21037/apm-21-3210. PMID: 35016480

Clinical prediction guides

Nakagawa N, Ishiyama K, Usuki K, Takada S, Tomikawa T, Handa H, Katsuoka Y, Hirano D, Sezaki N, Sumi M, Fujisawa S, Taniguchi Y, Mugitani A, Yoshimura T, Ohtsuka E, Takase K, Suehiro Y, Ota S, Kajiguchi T, Maeda T, Yamamoto M, Ohtake S, Katsumi A, Kiyoi H, Matsumura I, Miyazaki Y
Ann Hematol 2024 Jan;103(1):307-320. Epub 2023 Nov 8 doi: 10.1007/s00277-023-05527-5. PMID: 37940714
Bernués M, González T, Corchete LA, Santos S, Durán MA, López-Andrade B, Riso LL, Martínez-Serra J, Ramos R, Iglesias J, Royo I, Rosell J
Cancer Genet 2022 Jun;264-265:60-65. Epub 2022 Mar 26 doi: 10.1016/j.cancergen.2022.03.004. PMID: 35397254
Yan W, Qu XY, Li H, Li YC, Li Y, Yao K, Yang W, Wang HH
Ann Palliat Med 2021 Dec;10(12):12431-12440. doi: 10.21037/apm-21-3210. PMID: 35016480
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
Wang SA, Patel KP, Pozdnyakova O, Peng J, Zuo Z, Dal Cin P, Steensma DP, Hasserjian RP
Mod Pathol 2016 Oct;29(10):1221-31. Epub 2016 Jul 15 doi: 10.1038/modpathol.2016.118. PMID: 27443511

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