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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

Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management.
Tefferi A, Vannucchi AM, Barbui T
Am J Hematol 2024 Apr;99(4):697-718. Epub 2024 Jan 25 doi: 10.1002/ajh.27216. PMID: 38269572
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

Recent clinical studies

Etiology

Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management.
Tefferi A, Vannucchi AM, Barbui T
Am J Hematol 2024 Apr;99(4):697-718. Epub 2024 Jan 25 doi: 10.1002/ajh.27216. PMID: 38269572
Clinical characteristics of Japanese patients with myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis.
Edahiro Y, Ochiai T, Hashimoto Y, Morishita S, Shirane S, Inano T, Furuya C, Koike M, Noguchi M, Usuki K, Shiratsuchi M, Nakajima K, Ohtsuka E, Tanaka H, Kawata E, Nakamae M, Ueda Y, Aota Y, Sugita Y, Ohara S, Yamasaki S, Asagoe K, Yoshida S, Yamanouchi J, Suzuki S, Kondo T, Kanisawa Y, Toyama K, Omura H, Mizuchi D, Sakamaki S, Ando M, Komatsu N
Int J Hematol 2023 Jul;118(1):47-53. Epub 2023 Apr 14 doi: 10.1007/s12185-023-03592-0. PMID: 37058247
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

Diagnosis

Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management.
Tefferi A, Vannucchi AM, Barbui T
Am J Hematol 2024 Apr;99(4):697-718. Epub 2024 Jan 25 doi: 10.1002/ajh.27216. PMID: 38269572
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

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
Myelodysplastic syndrome/myeloproliferative neoplasm overlap syndromes: a focused review.
Patnaik MM, Lasho T
Hematology Am Soc Hematol Educ Program 2020 Dec 4;2020(1):460-464. doi: 10.1182/hematology.2020000163. PMID: 33275673Free PMC Article
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
Refractory anemia with ring sideroblasts and RARS with thrombocytosis.
Patnaik MM, Tefferi A
Am J Hematol 2015 Jun;90(6):549-59. doi: 10.1002/ajh.24038. PMID: 25899435

Prognosis

Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management.
Tefferi A, Vannucchi AM, Barbui T
Am J Hematol 2024 Apr;99(4):697-718. Epub 2024 Jan 25 doi: 10.1002/ajh.27216. PMID: 38269572
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
Myelodysplastic syndrome/myeloproliferative neoplasm overlap syndromes: a focused review.
Patnaik MM, Lasho T
Hematology Am Soc Hematol Educ Program 2020 Dec 4;2020(1):460-464. doi: 10.1182/hematology.2020000163. PMID: 33275673Free 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

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
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
Refractory anemia with ring sideroblasts and RARS with thrombocytosis.
Patnaik MM, Tefferi A
Am J Hematol 2015 Jun;90(6):549-59. doi: 10.1002/ajh.24038. PMID: 25899435

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