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Myelodysplastic Syndrome with Ring Sideroblasts and Multilineage Dysplasia

MedGen UID:
226982
•Concept ID:
C1301356
•
Neoplastic Process
Synonyms: Myelodysplastic syndrome with ring sideroblasts and multilineage dysplasia; Refractory Cytopenia with Multilineage Dysplasia and Ring Sideroblasts
SNOMED CT: Myelodysplastic syndrome with ring sideroblasts and multilineage dysplasia (1260217004)

Definition

A myelodysplastic syndrome characterized by bi-cytopenia or pancytopenia, dysplastic changes in 10% or more of the cells in two or more of the myeloid cell lines, and 15% or more ring sideroblasts in the bone marrow. (WHO, 2001) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMyelodysplastic Syndrome with Ring Sideroblasts and Multilineage Dysplasia

Recent clinical studies

Etiology

Myelodysplastic syndromes with ring sideroblasts (MDS-RS) and MDS/myeloproliferative neoplasm with RS and thrombocytosis (MDS/MPN-RS-T) - "2021 update on diagnosis, risk-stratification, and management".
Patnaik MM, Tefferi A
Am J Hematol 2021 Mar 1;96(3):379-394. Epub 2021 Jan 28 doi: 10.1002/ajh.26090. PMID: 33428785
SF3B1 Mutation but Not Ring Sideroblasts Identifies a Specific Group of Myelodysplastic Syndrome-Refractory Cytopenia With Multilineage Dysplasia.
Xiong B, Xue M, Yu Y, Wu S, Zuo X
Clin Lymphoma Myeloma Leuk 2020 May;20(5):329-339.e3. Epub 2020 Jan 16 doi: 10.1016/j.clml.2020.01.005. PMID: 32037286
Refractory anemia with ring sideroblasts (RARS) and RARS with thrombocytosis: "2019 Update on Diagnosis, Risk-stratification, and Management".
Patnaik MM, Tefferi A
Am J Hematol 2019 Apr;94(4):475-488. Epub 2019 Jan 24 doi: 10.1002/ajh.25397. PMID: 30618061Free PMC Article
Refractory anemia with ring sideroblasts (RARS) and RARS with thrombocytosis (RARS-T): 2017 update on diagnosis, risk-stratification, and management.
Patnaik MM, Tefferi A
Am J Hematol 2017 Mar;92(3):297-310. doi: 10.1002/ajh.24637. PMID: 28188970Free PMC Article
Refractory anemia with ring sideroblasts.
Malcovati L, Cazzola M
Best Pract Res Clin Haematol 2013 Dec;26(4):377-85. Epub 2013 Oct 1 doi: 10.1016/j.beha.2013.09.005. PMID: 24507814

Diagnosis

Impact of the International Consensus Classification of myelodysplastic syndromes.
Zhang Y, Wu J, Xu Z, Qin T, Qu S, Pan L, Wang H, Sun Q, Li C, Jia Y, Cai W, Yan X, Gong J, Gao Q, Li B, Gale RP, Xiao Z
Br J Haematol 2023 May;201(3):443-448. Epub 2022 Dec 28 doi: 10.1111/bjh.18628. PMID: 36575970
Myelodysplastic syndromes with ring sideroblasts (MDS-RS) and MDS/myeloproliferative neoplasm with RS and thrombocytosis (MDS/MPN-RS-T) - "2021 update on diagnosis, risk-stratification, and management".
Patnaik MM, Tefferi A
Am J Hematol 2021 Mar 1;96(3):379-394. Epub 2021 Jan 28 doi: 10.1002/ajh.26090. PMID: 33428785
SF3B1 Mutation but Not Ring Sideroblasts Identifies a Specific Group of Myelodysplastic Syndrome-Refractory Cytopenia With Multilineage Dysplasia.
Xiong B, Xue M, Yu Y, Wu S, Zuo X
Clin Lymphoma Myeloma Leuk 2020 May;20(5):329-339.e3. Epub 2020 Jan 16 doi: 10.1016/j.clml.2020.01.005. PMID: 32037286
Refractory anemia with ring sideroblasts (RARS) and RARS with thrombocytosis: "2019 Update on Diagnosis, Risk-stratification, and Management".
Patnaik MM, Tefferi A
Am J Hematol 2019 Apr;94(4):475-488. Epub 2019 Jan 24 doi: 10.1002/ajh.25397. PMID: 30618061Free PMC Article
Recent advances in the understanding of myelodysplastic syndromes with ring sideroblasts.
Malcovati L, Cazzola M
Br J Haematol 2016 Sep;174(6):847-58. Epub 2016 Jul 8 doi: 10.1111/bjh.14215. PMID: 27391606

Therapy

Myelodysplastic syndromes with ring sideroblasts (MDS-RS) and MDS/myeloproliferative neoplasm with RS and thrombocytosis (MDS/MPN-RS-T) - "2021 update on diagnosis, risk-stratification, and management".
Patnaik MM, Tefferi A
Am J Hematol 2021 Mar 1;96(3):379-394. Epub 2021 Jan 28 doi: 10.1002/ajh.26090. PMID: 33428785
SF3B1 Mutation but Not Ring Sideroblasts Identifies a Specific Group of Myelodysplastic Syndrome-Refractory Cytopenia With Multilineage Dysplasia.
Xiong B, Xue M, Yu Y, Wu S, Zuo X
Clin Lymphoma Myeloma Leuk 2020 May;20(5):329-339.e3. Epub 2020 Jan 16 doi: 10.1016/j.clml.2020.01.005. PMID: 32037286
Diagnosis and treatment of sideroblastic anemias: from defective heme synthesis to abnormal RNA splicing.
Cazzola M, Malcovati L
Hematology Am Soc Hematol Educ Program 2015;2015:19-25. doi: 10.1182/asheducation-2015.1.19. PMID: 26637696
Clinical and genetic characteristics of congenital sideroblastic anemia: comparison with myelodysplastic syndrome with ring sideroblast (MDS-RS).
Ohba R, Furuyama K, Yoshida K, Fujiwara T, Fukuhara N, Onishi Y, Manabe A, Ito E, Ozawa K, Kojima S, Ogawa S, Harigae H
Ann Hematol 2013 Jan;92(1):1-9. Epub 2012 Sep 16 doi: 10.1007/s00277-012-1564-5. PMID: 22983749Free PMC Article
Therapy-related myeloid neoplasms following treatment with radioiodine.
Schroeder T, Kuendgen A, Kayser S, Kröger N, Braulke F, Platzbecker U, Klärner V, Zohren F, Haase D, Stadler M, Schlenk R, Czibere AG, Bruns I, Fenk R, Gattermann N, Haas R, Kobbe G, Germing U
Haematologica 2012 Feb;97(2):206-12. Epub 2011 Oct 11 doi: 10.3324/haematol.2011.049114. PMID: 21993688Free PMC Article

Prognosis

Myelodysplastic syndromes with ring sideroblasts (MDS-RS) and MDS/myeloproliferative neoplasm with RS and thrombocytosis (MDS/MPN-RS-T) - "2021 update on diagnosis, risk-stratification, and management".
Patnaik MM, Tefferi A
Am J Hematol 2021 Mar 1;96(3):379-394. Epub 2021 Jan 28 doi: 10.1002/ajh.26090. PMID: 33428785
SF3B1 Mutation but Not Ring Sideroblasts Identifies a Specific Group of Myelodysplastic Syndrome-Refractory Cytopenia With Multilineage Dysplasia.
Xiong B, Xue M, Yu Y, Wu S, Zuo X
Clin Lymphoma Myeloma Leuk 2020 May;20(5):329-339.e3. Epub 2020 Jan 16 doi: 10.1016/j.clml.2020.01.005. PMID: 32037286
Refractory anemia with ring sideroblasts (RARS) and RARS with thrombocytosis (RARS-T): 2017 update on diagnosis, risk-stratification, and management.
Patnaik MM, Tefferi A
Am J Hematol 2017 Mar;92(3):297-310. doi: 10.1002/ajh.24637. PMID: 28188970Free PMC Article
Recent advances in the understanding of myelodysplastic syndromes with ring sideroblasts.
Malcovati L, Cazzola M
Br J Haematol 2016 Sep;174(6):847-58. Epub 2016 Jul 8 doi: 10.1111/bjh.14215. PMID: 27391606
Refractory anemia with ring sideroblasts.
Malcovati L, Cazzola M
Best Pract Res Clin Haematol 2013 Dec;26(4):377-85. Epub 2013 Oct 1 doi: 10.1016/j.beha.2013.09.005. PMID: 24507814

Clinical prediction guides

Prognostic impact of ASXL1 mutations in patients with myelodysplastic syndromes and multilineage dysplasia with or without ring sideroblasts.
Mangaonkar AA, Gangat N, Al-Kali A, Elliott MA, Begna KH, Hanson CA, Ketterling RP, Wolanskyj-Spinner AP, Hogan WJ, Litzow MR, Patnaik MM
Leuk Res 2018 Aug;71:60-62. Epub 2018 Jul 11 doi: 10.1016/j.leukres.2018.07.010. PMID: 30015104
Dynamics of DNMT3A mutation and prognostic relevance in patients with primary myelodysplastic syndrome.
Lin ME, Hou HA, Tsai CH, Wu SJ, Kuo YY, Tseng MH, Liu MC, Liu CW, Chou WC, Chen CY, Tang JL, Yao M, Li CC, Huang SY, Ko BS, Hsu SC, Lin CT, Tien HF
Clin Epigenetics 2018;10:42. Epub 2018 Apr 2 doi: 10.1186/s13148-018-0476-1. PMID: 29619119Free PMC Article
Refractory anemia with ring sideroblasts (RARS) and RARS with thrombocytosis (RARS-T): 2017 update on diagnosis, risk-stratification, and management.
Patnaik MM, Tefferi A
Am J Hematol 2017 Mar;92(3):297-310. doi: 10.1002/ajh.24637. PMID: 28188970Free PMC Article
SF3B1 mutation identifies a distinct subset of myelodysplastic syndrome with ring sideroblasts.
Malcovati L, Karimi M, Papaemmanuil E, Ambaglio I, Jädersten M, Jansson M, Elena C, Gallì A, Walldin G, Della Porta MG, Raaschou-Jensen K, Travaglino E, Kallenbach K, Pietra D, Ljungström V, Conte S, Boveri E, Invernizzi R, Rosenquist R, Campbell PJ, Cazzola M, Hellström Lindberg E
Blood 2015 Jul 9;126(2):233-41. Epub 2015 May 8 doi: 10.1182/blood-2015-03-633537. PMID: 25957392Free PMC Article
Myelodysplastic syndrome in children: differentiation from acute myeloid leukemia with a low blast count.
Chan GC, Wang WC, Raimondi SC, Behm FG, Krance RA, Chen G, Freiberg A, Ingram L, Butler D, Head DR
Leukemia 1997 Feb;11(2):206-11. doi: 10.1038/sj.leu.2400558. PMID: 9009082

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