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Post-Essential Thrombocythemia Myelofibrosis

MedGen UID:
923817
•Concept ID:
C3805233
•
Neoplastic Process
Synonyms: Myelofibrosis due to and following essential thrombocythemia; Myelofibrosis Transformation in Essential Thrombocythemia; Post-essential thrombocythemia myelofibrosis; Post-ET MF; 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  
  • CROGVPost-Essential Thrombocythemia Myelofibrosis

Professional guidelines

PubMed

Essential Thrombocythemia and Post-Essential Thrombocythemia Myelofibrosis: Updates on Diagnosis, Clinical Aspects, and Management.
Puglianini OC, Peker D, Zhang L, Papadantonakis N
Lab Med 2023 Jan 5;54(1):13-22. doi: 10.1093/labmed/lmac074. PMID: 35960786
Ruxolitinib therapy for myelofibrosis in Austria : Consensus on therapy management.
Krauth MT, Burgstaller S, Buxhofer-Ausch V, Gastl G, Geissler K, Keil F, Krippl P, Melchardt T, Petzer A, Rumpold H, Sliwa T, Wöhrer S, Wölfler A, Gisslinger H
Wien Klin Wochenschr 2018 Sep;130(17-18):495-504. Epub 2018 Jul 24 doi: 10.1007/s00508-018-1365-5. PMID: 30043249Free PMC Article
Emerging drugs for the treatment of Myelofibrosis.
Shreenivas A, Mascarenhas J
Expert Opin Emerg Drugs 2018 Mar;23(1):37-49. Epub 2018 Mar 6 doi: 10.1080/14728214.2018.1445718. PMID: 29480036

Recent clinical studies

Therapy

Pacritinib: First Approval.
Lamb YN
Drugs 2022 May;82(7):831-838. doi: 10.1007/s40265-022-01718-y. PMID: 35567653
Fedratinib: First Approval.
Blair HA
Drugs 2019 Oct;79(15):1719-1725. doi: 10.1007/s40265-019-01205-x. PMID: 31571162
Emerging drugs for the treatment of Myelofibrosis.
Shreenivas A, Mascarenhas J
Expert Opin Emerg Drugs 2018 Mar;23(1):37-49. Epub 2018 Mar 6 doi: 10.1080/14728214.2018.1445718. PMID: 29480036
A clinical-molecular prognostic model to predict survival in patients with post polycythemia vera and post essential thrombocythemia myelofibrosis.
Passamonti F, Giorgino T, Mora B, Guglielmelli P, Rumi E, Maffioli M, Rambaldi A, Caramella M, Komrokji R, Gotlib J, Kiladjian JJ, Cervantes F, Devos T, Palandri F, De Stefano V, Ruggeri M, Silver RT, Benevolo G, Albano F, Caramazza D, Merli M, Pietra D, Casalone R, Rotunno G, Barbui T, Cazzola M, Vannucchi AM
Leukemia 2017 Dec;31(12):2726-2731. Epub 2017 May 31 doi: 10.1038/leu.2017.169. PMID: 28561069
JAK inhibition with ruxolitinib versus best available therapy for myelofibrosis.
Harrison C, Kiladjian JJ, Al-Ali HK, Gisslinger H, Waltzman R, Stalbovskaya V, McQuitty M, Hunter DS, Levy R, Knoops L, Cervantes F, Vannucchi AM, Barbui T, Barosi G
N Engl J Med 2012 Mar 1;366(9):787-98. doi: 10.1056/NEJMoa1110556. PMID: 22375970

Prognosis

Real-world clinical characteristics of post-essential thrombocythemia and post-polycythemia vera myelofibrosis.
Shide K, Takenaka K, Kitanaka A, Numata A, Kameda T, Yamauchi T, Inagaki A, Mizuno S, Takami A, Ito S, Hagihara M, Usuki K, Maekawa T, Sunami K, Ueda Y, Tsutsui M, Ando M, Komatsu N, Ozawa K, Kurokawa M, Arai S, Mitani K, Akashi K, Shimoda K
Ann Hematol 2024 Jan;103(1):97-103. Epub 2023 Nov 10 doi: 10.1007/s00277-023-05528-4. PMID: 37946031
Essential Thrombocythemia and Post-Essential Thrombocythemia Myelofibrosis: Updates on Diagnosis, Clinical Aspects, and Management.
Puglianini OC, Peker D, Zhang L, Papadantonakis N
Lab Med 2023 Jan 5;54(1):13-22. doi: 10.1093/labmed/lmac074. PMID: 35960786
Emerging drugs for the treatment of Myelofibrosis.
Shreenivas A, Mascarenhas J
Expert Opin Emerg Drugs 2018 Mar;23(1):37-49. Epub 2018 Mar 6 doi: 10.1080/14728214.2018.1445718. PMID: 29480036
A clinical-molecular prognostic model to predict survival in patients with post polycythemia vera and post essential thrombocythemia myelofibrosis.
Passamonti F, Giorgino T, Mora B, Guglielmelli P, Rumi E, Maffioli M, Rambaldi A, Caramella M, Komrokji R, Gotlib J, Kiladjian JJ, Cervantes F, Devos T, Palandri F, De Stefano V, Ruggeri M, Silver RT, Benevolo G, Albano F, Caramazza D, Merli M, Pietra D, Casalone R, Rotunno G, Barbui T, Cazzola M, Vannucchi AM
Leukemia 2017 Dec;31(12):2726-2731. Epub 2017 May 31 doi: 10.1038/leu.2017.169. PMID: 28561069
JAK inhibition with ruxolitinib versus best available therapy for myelofibrosis.
Harrison C, Kiladjian JJ, Al-Ali HK, Gisslinger H, Waltzman R, Stalbovskaya V, McQuitty M, Hunter DS, Levy R, Knoops L, Cervantes F, Vannucchi AM, Barbui T, Barosi G
N Engl J Med 2012 Mar 1;366(9):787-98. doi: 10.1056/NEJMoa1110556. PMID: 22375970

Clinical prediction guides

Essential Thrombocythemia and Post-Essential Thrombocythemia Myelofibrosis: Updates on Diagnosis, Clinical Aspects, and Management.
Puglianini OC, Peker D, Zhang L, Papadantonakis N
Lab Med 2023 Jan 5;54(1):13-22. doi: 10.1093/labmed/lmac074. PMID: 35960786
Prediction of thrombosis in post-polycythemia vera and post-essential thrombocythemia myelofibrosis: a study on 1258 patients.
Mora B, Guglielmelli P, Kuykendall A, Rumi E, Maffioli M, Palandri F, De Stefano V, Caramella M, Salmoiraghi S, Kiladjian JJ, Gotlib J, Iurlo A, Cervantes F, Ruggeri M, Silver RT, Albano F, Benevolo G, Ross DM, Della Porta MG, Devos T, Rotunno G, Komrokji RS, Casetti IC, Merli M, Brociner M, Caramazza D, Auteri G, Barbui T, Cattaneo D, Bertù L, Arcaini L, Vannucchi AM, Passamonti F
Leukemia 2022 Oct;36(10):2453-2460. Epub 2022 Aug 30 doi: 10.1038/s41375-022-01673-3. PMID: 36042316
The evolving understanding of prognosis in post-essential thrombocythemia myelofibrosis and post-polycythemia vera myelofibrosis vs primary myelofibrosis.
Masarova L, Verstovsek S
Clin Adv Hematol Oncol 2019 May;17(5):299-307. PMID: 31188809
A clinical-molecular prognostic model to predict survival in patients with post polycythemia vera and post essential thrombocythemia myelofibrosis.
Passamonti F, Giorgino T, Mora B, Guglielmelli P, Rumi E, Maffioli M, Rambaldi A, Caramella M, Komrokji R, Gotlib J, Kiladjian JJ, Cervantes F, Devos T, Palandri F, De Stefano V, Ruggeri M, Silver RT, Benevolo G, Albano F, Caramazza D, Merli M, Pietra D, Casalone R, Rotunno G, Barbui T, Cazzola M, Vannucchi AM
Leukemia 2017 Dec;31(12):2726-2731. Epub 2017 May 31 doi: 10.1038/leu.2017.169. PMID: 28561069
The evolution and clinical relevance of prognostic classification systems in myelofibrosis.
Bose P, Verstovsek S
Cancer 2016 Mar 1;122(5):681-92. Epub 2015 Dec 30 doi: 10.1002/cncr.29842. PMID: 26717494

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