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Myelofibrosis Transformation in Essential Thrombocythemia

MedGen UID:
923817
•Concept ID:
C3805233
•
Neoplastic Process
Synonyms: Myelofibrosis due to and following essential thrombocythemia; Post Essential Thrombocythemia Myelofibrosis; Post-Essential Thrombocythemia Myelofibrosis; Post-essential thrombocythemia myelofibrosis; Post-Essential Thrombocytosis Myelofibrosis; Post-ET Myelofibrosis
SNOMED CT: Post-essential thrombocythemia myelofibrosis (871541008); Myelofibrosis due to and following essential thrombocythemia (871541008)

Definition

Myelofibrosis that develops in a patient with history of essential thrombocythemia. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMyelofibrosis Transformation in Essential Thrombocythemia

Professional guidelines

PubMed

Integration of Molecular Information in Risk Assessment of Patients with Myeloproliferative Neoplasms.
Loscocco GG, Coltro G, Guglielmelli P, Vannucchi AM
Cells 2021 Aug 2;10(8) doi: 10.3390/cells10081962. PMID: 34440731Free PMC Article
Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2020 Dec;95(12):1599-1613. Epub 2020 Oct 23 doi: 10.1002/ajh.26008. PMID: 32974939
Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2019 Jan;94(1):133-143. Epub 2018 Nov 9 doi: 10.1002/ajh.25303. PMID: 30281843

Recent clinical studies

Etiology

Progression of Myeloproliferative Neoplasms (MPN): Diagnostic and Therapeutic Perspectives.
Baumeister J, Chatain N, Sofias AM, Lammers T, Koschmieder S
Cells 2021 Dec 16;10(12) doi: 10.3390/cells10123551. PMID: 34944059Free PMC Article
Molecular pathogenesis of the myeloproliferative neoplasms.
Greenfield G, McMullin MF, Mills K
J Hematol Oncol 2021 Jun 30;14(1):103. doi: 10.1186/s13045-021-01116-z. PMID: 34193229Free PMC Article
Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2020 Dec;95(12):1599-1613. Epub 2020 Oct 23 doi: 10.1002/ajh.26008. PMID: 32974939
Classification and Personalized Prognosis in Myeloproliferative Neoplasms.
Grinfeld J, Nangalia J, Baxter EJ, Wedge DC, Angelopoulos N, Cantrill R, Godfrey AL, Papaemmanuil E, Gundem G, MacLean C, Cook J, O'Neil L, O'Meara S, Teague JW, Butler AP, Massie CE, Williams N, Nice FL, Andersen CL, Hasselbalch HC, Guglielmelli P, McMullin MF, Vannucchi AM, Harrison CN, Gerstung M, Green AR, Campbell PJ
N Engl J Med 2018 Oct 11;379(15):1416-1430. doi: 10.1056/NEJMoa1716614. PMID: 30304655Free PMC Article
Myeloproliferative Neoplasms: A Contemporary Review.
Tefferi A, Pardanani A
JAMA Oncol 2015 Apr;1(1):97-105. doi: 10.1001/jamaoncol.2015.89. PMID: 26182311

Diagnosis

Progression of Myeloproliferative Neoplasms (MPN): Diagnostic and Therapeutic Perspectives.
Baumeister J, Chatain N, Sofias AM, Lammers T, Koschmieder S
Cells 2021 Dec 16;10(12) doi: 10.3390/cells10123551. PMID: 34944059Free PMC Article
Molecular pathogenesis of the myeloproliferative neoplasms.
Greenfield G, McMullin MF, Mills K
J Hematol Oncol 2021 Jun 30;14(1):103. doi: 10.1186/s13045-021-01116-z. PMID: 34193229Free PMC Article
Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2020 Dec;95(12):1599-1613. Epub 2020 Oct 23 doi: 10.1002/ajh.26008. PMID: 32974939
Classification and Personalized Prognosis in Myeloproliferative Neoplasms.
Grinfeld J, Nangalia J, Baxter EJ, Wedge DC, Angelopoulos N, Cantrill R, Godfrey AL, Papaemmanuil E, Gundem G, MacLean C, Cook J, O'Neil L, O'Meara S, Teague JW, Butler AP, Massie CE, Williams N, Nice FL, Andersen CL, Hasselbalch HC, Guglielmelli P, McMullin MF, Vannucchi AM, Harrison CN, Gerstung M, Green AR, Campbell PJ
N Engl J Med 2018 Oct 11;379(15):1416-1430. doi: 10.1056/NEJMoa1716614. PMID: 30304655Free PMC Article
Myeloproliferative Neoplasms: A Contemporary Review.
Tefferi A, Pardanani A
JAMA Oncol 2015 Apr;1(1):97-105. doi: 10.1001/jamaoncol.2015.89. PMID: 26182311

Therapy

Novel therapeutics in myeloproliferative neoplasms.
Venugopal S, Mascarenhas J
J Hematol Oncol 2020 Dec 2;13(1):162. doi: 10.1186/s13045-020-00995-y. PMID: 33267911Free PMC Article
Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2020 Dec;95(12):1599-1613. Epub 2020 Oct 23 doi: 10.1002/ajh.26008. PMID: 32974939
Polycythemia vera and essential thrombocythemia: 2019 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2019 Jan;94(1):133-143. Epub 2018 Nov 9 doi: 10.1002/ajh.25303. PMID: 30281843
Myeloproliferative Neoplasms: A Contemporary Review.
Tefferi A, Pardanani A
JAMA Oncol 2015 Apr;1(1):97-105. doi: 10.1001/jamaoncol.2015.89. PMID: 26182311
Essential thrombocythemia.
Finazzi G, Harrison C
Semin Hematol 2005 Oct;42(4):230-8. doi: 10.1053/j.seminhematol.2005.05.022. PMID: 16210036

Prognosis

Molecular pathogenesis of the myeloproliferative neoplasms.
Greenfield G, McMullin MF, Mills K
J Hematol Oncol 2021 Jun 30;14(1):103. doi: 10.1186/s13045-021-01116-z. PMID: 34193229Free PMC Article
Inflammatory Pathophysiology as a Contributor to Myeloproliferative Neoplasms.
Fisher DAC, Fowles JS, Zhou A, Oh ST
Front Immunol 2021;12:683401. Epub 2021 Jun 1 doi: 10.3389/fimmu.2021.683401. PMID: 34140953Free PMC Article
Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management.
Tefferi A, Barbui T
Am J Hematol 2020 Dec;95(12):1599-1613. Epub 2020 Oct 23 doi: 10.1002/ajh.26008. PMID: 32974939
Classification and Personalized Prognosis in Myeloproliferative Neoplasms.
Grinfeld J, Nangalia J, Baxter EJ, Wedge DC, Angelopoulos N, Cantrill R, Godfrey AL, Papaemmanuil E, Gundem G, MacLean C, Cook J, O'Neil L, O'Meara S, Teague JW, Butler AP, Massie CE, Williams N, Nice FL, Andersen CL, Hasselbalch HC, Guglielmelli P, McMullin MF, Vannucchi AM, Harrison CN, Gerstung M, Green AR, Campbell PJ
N Engl J Med 2018 Oct 11;379(15):1416-1430. doi: 10.1056/NEJMoa1716614. PMID: 30304655Free PMC Article
Myeloproliferative Neoplasms: A Contemporary Review.
Tefferi A, Pardanani A
JAMA Oncol 2015 Apr;1(1):97-105. doi: 10.1001/jamaoncol.2015.89. PMID: 26182311

Clinical prediction guides

Real-world study of children and young adults with myeloproliferative neoplasms: identifying risks and unmet needs.
Sobas M, Kiladjian JJ, Beauverd Y, Curto-Garcia N, Sadjadian P, Shih LY, Devos T, Krochmalczyk D, Galli S, Bieniaszewska M, Seferynska I, McMullin MF, Armatys A, Spalek A, Waclaw J, Zdrenghea M, Legros L, Girodon F, Lewandowski K, Angona Figueras A, Samuelsson J, Abuin Blanco A, Cony-Makhoul P, Collins A, James C, Kusec R, Lauermannova M, Noya MS, Skowronek M, Szukalski L, Szmigielska-Kaplon A, Wondergem M, Dudchenko I, Gora Tybor J, Laribi K, Kulikowska de Nalecz A, Demory JL, Le Du K, Zweegman S, Besses Raebel C, Skoda R, Giraudier S, Griesshammer M, Harrison CN, Ianotto JC
Blood Adv 2022 Sep 13;6(17):5171-5183. doi: 10.1182/bloodadvances.2022007201. PMID: 35802458Free PMC Article
Myelofibrosis: Genetic Characteristics and the Emerging Therapeutic Landscape.
Tefferi A, Gangat N, Pardanani A, Crispino JD
Cancer Res 2022 Mar 1;82(5):749-763. doi: 10.1158/0008-5472.CAN-21-2930. PMID: 34911786Free PMC Article
Inflammatory Pathophysiology as a Contributor to Myeloproliferative Neoplasms.
Fisher DAC, Fowles JS, Zhou A, Oh ST
Front Immunol 2021;12:683401. Epub 2021 Jun 1 doi: 10.3389/fimmu.2021.683401. PMID: 34140953Free PMC Article
Classification and Personalized Prognosis in Myeloproliferative Neoplasms.
Grinfeld J, Nangalia J, Baxter EJ, Wedge DC, Angelopoulos N, Cantrill R, Godfrey AL, Papaemmanuil E, Gundem G, MacLean C, Cook J, O'Neil L, O'Meara S, Teague JW, Butler AP, Massie CE, Williams N, Nice FL, Andersen CL, Hasselbalch HC, Guglielmelli P, McMullin MF, Vannucchi AM, Harrison CN, Gerstung M, Green AR, Campbell PJ
N Engl J Med 2018 Oct 11;379(15):1416-1430. doi: 10.1056/NEJMoa1716614. PMID: 30304655Free PMC Article
Myeloproliferative Neoplasms: A Contemporary Review.
Tefferi A, Pardanani A
JAMA Oncol 2015 Apr;1(1):97-105. doi: 10.1001/jamaoncol.2015.89. PMID: 26182311

Recent systematic reviews

A systematic review and meta-analysis of the prevalence of thrombosis and bleeding at diagnosis of Philadelphia-negative myeloproliferative neoplasms.
Rungjirajittranon T, Owattanapanich W, Ungprasert P, Siritanaratkul N, Ruchutrakool T
BMC Cancer 2019 Feb 28;19(1):184. doi: 10.1186/s12885-019-5387-9. PMID: 30819138Free PMC Article

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