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Refractory Primary Myelofibrosis

MedGen UID:
1740721
•Concept ID:
C5419092
•
Neoplastic Process

Definition

Primary myelofibrosis that is resistant to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRefractory Primary Myelofibrosis

Professional guidelines

PubMed

Primary myelofibrosis: 2023 update on diagnosis, risk-stratification, and management.
Tefferi A
Am J Hematol 2023 May;98(5):801-821. Epub 2023 Feb 6 doi: 10.1002/ajh.26857. PMID: 36680511
Next Generation Therapeutics for the Treatment of Myelofibrosis.
Tremblay D, Mascarenhas J
Cells 2021 Apr 27;10(5) doi: 10.3390/cells10051034. PMID: 33925695Free PMC Article
Primary myelofibrosis: 2021 update on diagnosis, risk-stratification and management.
Tefferi A
Am J Hematol 2021 Jan;96(1):145-162. Epub 2020 Dec 2 doi: 10.1002/ajh.26050. PMID: 33197049

Recent clinical studies

Etiology

Primary myelofibrosis: 2023 update on diagnosis, risk-stratification, and management.
Tefferi A
Am J Hematol 2023 May;98(5):801-821. Epub 2023 Feb 6 doi: 10.1002/ajh.26857. PMID: 36680511
Primary myelofibrosis: 2021 update on diagnosis, risk-stratification and management.
Tefferi A
Am J Hematol 2021 Jan;96(1):145-162. Epub 2020 Dec 2 doi: 10.1002/ajh.26050. PMID: 33197049
Fedratinib, a newly approved treatment for patients with myeloproliferative neoplasm-associated myelofibrosis.
Talpaz M, Kiladjian JJ
Leukemia 2021 Jan;35(1):1-17. Epub 2020 Jul 9 doi: 10.1038/s41375-020-0954-2. PMID: 32647323Free PMC Article
Primary myelofibrosis: 2017 update on diagnosis, risk-stratification, and management.
Tefferi A
Am J Hematol 2016 Dec;91(12):1262-1271. doi: 10.1002/ajh.24592. PMID: 27870387
Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet.
Barbui T, Barosi G, Birgegard G, Cervantes F, Finazzi G, Griesshammer M, Harrison C, Hasselbalch HC, Hehlmann R, Hoffman R, Kiladjian JJ, Kröger N, Mesa R, McMullin MF, Pardanani A, Passamonti F, Vannucchi AM, Reiter A, Silver RT, Verstovsek S, Tefferi A; European LeukemiaNet
J Clin Oncol 2011 Feb 20;29(6):761-70. Epub 2011 Jan 4 doi: 10.1200/JCO.2010.31.8436. PMID: 21205761Free PMC Article

Diagnosis

Primary myelofibrosis: 2023 update on diagnosis, risk-stratification, and management.
Tefferi A
Am J Hematol 2023 May;98(5):801-821. Epub 2023 Feb 6 doi: 10.1002/ajh.26857. PMID: 36680511
Primary myelofibrosis: 2021 update on diagnosis, risk-stratification and management.
Tefferi A
Am J Hematol 2021 Jan;96(1):145-162. Epub 2020 Dec 2 doi: 10.1002/ajh.26050. PMID: 33197049
Fedratinib, a newly approved treatment for patients with myeloproliferative neoplasm-associated myelofibrosis.
Talpaz M, Kiladjian JJ
Leukemia 2021 Jan;35(1):1-17. Epub 2020 Jul 9 doi: 10.1038/s41375-020-0954-2. PMID: 32647323Free PMC Article
Primary myelofibrosis: 2017 update on diagnosis, risk-stratification, and management.
Tefferi A
Am J Hematol 2016 Dec;91(12):1262-1271. doi: 10.1002/ajh.24592. PMID: 27870387
Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet.
Barbui T, Barosi G, Birgegard G, Cervantes F, Finazzi G, Griesshammer M, Harrison C, Hasselbalch HC, Hehlmann R, Hoffman R, Kiladjian JJ, Kröger N, Mesa R, McMullin MF, Pardanani A, Passamonti F, Vannucchi AM, Reiter A, Silver RT, Verstovsek S, Tefferi A; European LeukemiaNet
J Clin Oncol 2011 Feb 20;29(6):761-70. Epub 2011 Jan 4 doi: 10.1200/JCO.2010.31.8436. PMID: 21205761Free PMC Article

Therapy

Primary myelofibrosis: 2023 update on diagnosis, risk-stratification, and management.
Tefferi A
Am J Hematol 2023 May;98(5):801-821. Epub 2023 Feb 6 doi: 10.1002/ajh.26857. PMID: 36680511
Next Generation Therapeutics for the Treatment of Myelofibrosis.
Tremblay D, Mascarenhas J
Cells 2021 Apr 27;10(5) doi: 10.3390/cells10051034. PMID: 33925695Free PMC Article
Fedratinib, a newly approved treatment for patients with myeloproliferative neoplasm-associated myelofibrosis.
Talpaz M, Kiladjian JJ
Leukemia 2021 Jan;35(1):1-17. Epub 2020 Jul 9 doi: 10.1038/s41375-020-0954-2. PMID: 32647323Free PMC Article
Splenectomy in Myelofibrosis: Indications, Efficacy, and Complications.
Malato A, Rossi E, Tiribelli M, Mendicino F, Pugliese N
Clin Lymphoma Myeloma Leuk 2020 Sep;20(9):588-595. Epub 2020 Apr 30 doi: 10.1016/j.clml.2020.04.015. PMID: 32482540
Primary myelofibrosis: 2013 update on diagnosis, risk-stratification, and management.
Tefferi A
Am J Hematol 2013 Feb;88(2):141-50. doi: 10.1002/ajh.23384. PMID: 23349007

Prognosis

Primary myelofibrosis: 2023 update on diagnosis, risk-stratification, and management.
Tefferi A
Am J Hematol 2023 May;98(5):801-821. Epub 2023 Feb 6 doi: 10.1002/ajh.26857. PMID: 36680511
Primary myelofibrosis: 2021 update on diagnosis, risk-stratification and management.
Tefferi A
Am J Hematol 2021 Jan;96(1):145-162. Epub 2020 Dec 2 doi: 10.1002/ajh.26050. PMID: 33197049
Primary myelofibrosis: 2017 update on diagnosis, risk-stratification, and management.
Tefferi A
Am J Hematol 2016 Dec;91(12):1262-1271. doi: 10.1002/ajh.24592. PMID: 27870387
Primary myelofibrosis: 2013 update on diagnosis, risk-stratification, and management.
Tefferi A
Am J Hematol 2013 Feb;88(2):141-50. doi: 10.1002/ajh.23384. PMID: 23349007
Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet.
Barbui T, Barosi G, Birgegard G, Cervantes F, Finazzi G, Griesshammer M, Harrison C, Hasselbalch HC, Hehlmann R, Hoffman R, Kiladjian JJ, Kröger N, Mesa R, McMullin MF, Pardanani A, Passamonti F, Vannucchi AM, Reiter A, Silver RT, Verstovsek S, Tefferi A; European LeukemiaNet
J Clin Oncol 2011 Feb 20;29(6):761-70. Epub 2011 Jan 4 doi: 10.1200/JCO.2010.31.8436. PMID: 21205761Free PMC Article

Clinical prediction guides

Primary myelofibrosis: 2023 update on diagnosis, risk-stratification, and management.
Tefferi A
Am J Hematol 2023 May;98(5):801-821. Epub 2023 Feb 6 doi: 10.1002/ajh.26857. PMID: 36680511
Primary myelofibrosis: 2021 update on diagnosis, risk-stratification and management.
Tefferi A
Am J Hematol 2021 Jan;96(1):145-162. Epub 2020 Dec 2 doi: 10.1002/ajh.26050. PMID: 33197049
Primary myelofibrosis: 2017 update on diagnosis, risk-stratification, and management.
Tefferi A
Am J Hematol 2016 Dec;91(12):1262-1271. doi: 10.1002/ajh.24592. PMID: 27870387
Primary myelofibrosis: 2013 update on diagnosis, risk-stratification, and management.
Tefferi A
Am J Hematol 2013 Feb;88(2):141-50. doi: 10.1002/ajh.23384. PMID: 23349007
Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet.
Barbui T, Barosi G, Birgegard G, Cervantes F, Finazzi G, Griesshammer M, Harrison C, Hasselbalch HC, Hehlmann R, Hoffman R, Kiladjian JJ, Kröger N, Mesa R, McMullin MF, Pardanani A, Passamonti F, Vannucchi AM, Reiter A, Silver RT, Verstovsek S, Tefferi A; European LeukemiaNet
J Clin Oncol 2011 Feb 20;29(6):761-70. Epub 2011 Jan 4 doi: 10.1200/JCO.2010.31.8436. PMID: 21205761Free PMC Article

Recent systematic reviews

Indication and management of allogeneic stem cell transplantation in primary myelofibrosis: a consensus process by an EBMT/ELN international working group.
Kröger NM, Deeg JH, Olavarria E, Niederwieser D, Bacigalupo A, Barbui T, Rambaldi A, Mesa R, Tefferi A, Griesshammer M, Gupta V, Harrison C, Alchalby H, Vannucchi AM, Cervantes F, Robin M, Ditschkowski M, Fauble V, McLornan D, Ballen K, Popat UR, Passamonti F, Rondelli D, Barosi G
Leukemia 2015 Nov;29(11):2126-33. Epub 2015 Aug 21 doi: 10.1038/leu.2015.233. PMID: 26293647

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