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Myelodysplastic/Myeloproliferative Neoplasm with Ring Sideroblasts and Thrombocytosis

MedGen UID:
414880
•Concept ID:
C2826330
•
Neoplastic Process
Synonyms: Essential Thrombocythemia with Ring Sideroblasts; MDS/MPN with Ring Sideroblasts and Thrombocytosis; Refractory Anemia with Ring Sideroblasts Associated with Marked Thrombocytosis

Definition

A myelodysplastic/myeloproliferative neoplasm with morphologic and clinical characteristics of refractory anemia with ring sideroblasts, marked thrombocytosis, and abnormal megakaryocytes. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMyelodysplastic/Myeloproliferative Neoplasm with Ring Sideroblasts and Thrombocytosis

Professional guidelines

PubMed

Atypical chronic myeloid leukemia and myelodysplastic/myeloproliferative neoplasm, not otherwise specified: 2023 update on diagnosis, risk stratification, and management.
Patnaik MM, Tefferi A
Am J Hematol 2023 Apr;98(4):681-689. Epub 2023 Jan 4 doi: 10.1002/ajh.26828. PMID: 36601682
Treatment outcomes for patients with myelodysplastic syndrome/myeloproliferative neoplasms with ring sideroblasts and thrombocytosis.
Komrokji R, Melody M, Al Ali N, Chan O, Klimek V, Ball BJ, Sekeres MA, Lucas G, Maciejewski JP, Sallman DA, Padron E, Kuykendall A, Lasho T, Al-Kali A, Naqvi K, Steensma DP, Garcia-Manero G, Patnaik MM
Leuk Lymphoma 2022 Jan;63(1):199-204. Epub 2021 Aug 27 doi: 10.1080/10428194.2021.1971217. PMID: 34448437
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

Recent clinical studies

Etiology

Atypical chronic myeloid leukemia and myelodysplastic/myeloproliferative neoplasm, not otherwise specified: 2023 update on diagnosis, risk stratification, and management.
Patnaik MM, Tefferi A
Am J Hematol 2023 Apr;98(4):681-689. Epub 2023 Jan 4 doi: 10.1002/ajh.26828. PMID: 36601682
Myelodysplastic syndrome/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis: Ringing in a new future.
Nathan DI, Feld J, El Jamal SM, Mascarenhas J, Tremblay D
Leuk Res 2022 Apr;115:106820. Epub 2022 Mar 7 doi: 10.1016/j.leukres.2022.106820. PMID: 35279478
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
Molecular genetics of MDS/MPN overlap syndromes.
Hunter AM, Padron E
Best Pract Res Clin Haematol 2020 Sep;33(3):101195. Epub 2020 Jul 5 doi: 10.1016/j.beha.2020.101195. PMID: 33038984
Challenges in Myelodysplastic/Myeloproliferative Neoplasms (MDS/MPN).
Smith BN, Savona M, Komrokji RS
Clin Lymphoma Myeloma Leuk 2019 Jan;19(1):1-8. Epub 2018 Nov 23 doi: 10.1016/j.clml.2018.11.019. PMID: 30555034Free PMC Article

Diagnosis

Atypical chronic myeloid leukemia and myelodysplastic/myeloproliferative neoplasm, not otherwise specified: 2023 update on diagnosis, risk stratification, and management.
Patnaik MM, Tefferi A
Am J Hematol 2023 Apr;98(4):681-689. Epub 2023 Jan 4 doi: 10.1002/ajh.26828. PMID: 36601682
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
Genomics of myelodysplastic syndrome/myeloproliferative neoplasm overlap syndromes.
Patnaik MM, Lasho TL
Hematology Am Soc Hematol Educ Program 2020 Dec 4;2020(1):450-459. doi: 10.1182/hematology.2020000130. PMID: 33275756Free PMC Article
Molecular landscape and clonal architecture of adult myelodysplastic/myeloproliferative neoplasms.
Palomo L, Meggendorfer M, Hutter S, Twardziok S, Ademà V, Fuhrmann I, Fuster-Tormo F, Xicoy B, Zamora L, Acha P, Kerr CM, Kern W, Maciejewski JP, Solé F, Haferlach C, Haferlach T
Blood 2020 Oct 15;136(16):1851-1862. doi: 10.1182/blood.2019004229. PMID: 32573691Free PMC Article
Myelodysplastic/myeloproliferative neoplasms.
Cazzola M, Malcovati L, Invernizzi R
Hematology Am Soc Hematol Educ Program 2011;2011:264-72. doi: 10.1182/asheducation-2011.1.264. PMID: 22160044

Therapy

New drugs for myeloid neoplasms with ring sideroblasts: Luspatercept vs imetelstat.
Tefferi A
Am J Hematol 2021 Jul 1;96(7):761-763. Epub 2021 May 3 doi: 10.1002/ajh.26197. PMID: 33861878
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
Clinical analysis and literature review of a case with the myelodysplastic syndrome/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis.
Long B, Shi H, Zhu C
Hematology 2020 Dec;25(1):283-285. doi: 10.1080/16078454.2020.1790840. PMID: 32657243

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
Genomics of myelodysplastic syndrome/myeloproliferative neoplasm overlap syndromes.
Patnaik MM, Lasho TL
Hematology Am Soc Hematol Educ Program 2020 Dec 4;2020(1):450-459. doi: 10.1182/hematology.2020000130. PMID: 33275756Free PMC Article
Molecular genetics of MDS/MPN overlap syndromes.
Hunter AM, Padron E
Best Pract Res Clin Haematol 2020 Sep;33(3):101195. Epub 2020 Jul 5 doi: 10.1016/j.beha.2020.101195. PMID: 33038984
Molecular landscape and clonal architecture of adult myelodysplastic/myeloproliferative neoplasms.
Palomo L, Meggendorfer M, Hutter S, Twardziok S, Ademà V, Fuhrmann I, Fuster-Tormo F, Xicoy B, Zamora L, Acha P, Kerr CM, Kern W, Maciejewski JP, Solé F, Haferlach C, Haferlach T
Blood 2020 Oct 15;136(16):1851-1862. doi: 10.1182/blood.2019004229. PMID: 32573691Free PMC Article
Myelodysplastic/myeloproliferative neoplasms.
Cazzola M, Malcovati L, Invernizzi R
Hematology Am Soc Hematol Educ Program 2011;2011:264-72. doi: 10.1182/asheducation-2011.1.264. PMID: 22160044

Clinical prediction guides

Clinical Application for Diagnosis of Myelodysplatic/Myeloproliferative Neoplasm with Ring Sideroblasts and Thrombocytosis.
Lee MY, Jang S, Park CJ, Cho YU
Clin Lab 2022 Apr 1;68(4) doi: 10.7754/Clin.Lab.2021.210738. PMID: 35443589
Myelodysplastic/myeloproliferative neoplasms with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T): Mayo-Moffitt collaborative study of 158 patients.
Mangaonkar AA, Lasho TL, Ketterling RP, Reichard KK, Gangat N, Al-Kali A, Begna KH, Pardanani A, Al Ali NH, Talati C, Sallman D, Padron E, Patnaik MM, Tefferi A, Komrokji R
Blood Cancer J 2022 Feb 1;12(2):26. doi: 10.1038/s41408-022-00622-8. PMID: 35105856Free PMC Article
Myelodysplastic/myeloproliferative neoplasms-unclassifiable with isolated isochromosome 17q represents a distinct clinico-biologic subset: a multi-institutional collaborative study from the Bone Marrow Pathology Group.
Kanagal-Shamanna R, Orazi A, Hasserjian RP, Arber DA, Reichard K, Hsi ED, Bagg A, Rogers HJ, Geyer J, Darbaniyan F, Do KA, Devins KM, Pozdnyakova O, George TI, Cin PD, Greipp PT, Routbort MJ, Patel K, Garcia-Manero G, Verstovsek S, Medeiros LJ, Wang SA, Bueso-Ramos C
Mod Pathol 2022 Apr;35(4):470-479. Epub 2021 Nov 13 doi: 10.1038/s41379-021-00961-0. PMID: 34775472Free PMC Article
Molecular landscape and clonal architecture of adult myelodysplastic/myeloproliferative neoplasms.
Palomo L, Meggendorfer M, Hutter S, Twardziok S, Ademà V, Fuhrmann I, Fuster-Tormo F, Xicoy B, Zamora L, Acha P, Kerr CM, Kern W, Maciejewski JP, Solé F, Haferlach C, Haferlach T
Blood 2020 Oct 15;136(16):1851-1862. doi: 10.1182/blood.2019004229. PMID: 32573691Free 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

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