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Cellular phase chronic idiopathic myelofibrosis

MedGen UID:
275741
•Concept ID:
C1516553
•
Neoplastic Process
Synonyms: cellular phase chronic idiopathic myelofibrosis; chronic idiopathic myelofibrosis, cellular phase; chronic idiopathic myelofibrosis, Prefibrotic stage; Early/Prefibrotic Primary Myelofibrosis; Early/Prefibrotic Stage PMF; Early/Prefibrotic Stage Primary Myelofibrosis; PMFPES; Pre-PMF; Prefibrotic/Early Primary Myelofibrosis; Prefibrotic/Early Primary myelofibrosis; Primary Myelofibrosis, Prefibrotic Stage; primary myelofibrosis, Prefibrotic stage; Primary Myelofibrosis, Prefibrotic/Early Stage; primary myelofibrosis, Prefibrotic/early stage
 
Monarch Initiative: MONDO:0004463

Definition

Primary myelofibrosis characterized by bone marrow hypercellularity and the presence of atypical megakaryocytes. There is no increase in the percentage of myeloblasts and no significant increase in reticulin or collagen fibrosis in the bone marrow. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCellular phase chronic idiopathic myelofibrosis

Professional guidelines

PubMed

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
Biology and clinical management of myeloproliferative neoplasms and development of the JAK inhibitor ruxolitinib.
Mascarenhas J, Mughal TI, Verstovsek S
Curr Med Chem 2012;19(26):4399-413. doi: 10.2174/092986712803251511. PMID: 22830345Free PMC Article
Biology and treatment of primary myelofibrosis.
Hoffman R, Rondelli D
Hematology Am Soc Hematol Educ Program 2007:346-54. doi: 10.1182/asheducation-2007.1.346. PMID: 18024650

Recent clinical studies

Etiology

Molecular Pathogenesis of Myeloproliferative Neoplasms.
Rolles B, Mullally A
Curr Hematol Malig Rep 2022 Dec;17(6):319-329. Epub 2022 Nov 7 doi: 10.1007/s11899-022-00685-1. PMID: 36336766
Circulating YKL-40 in Philadelphia-negative myeloproliferative neoplasms.
Krečak I, Gverić-Krečak V, Lapić I, Rončević P, Gulin J, Fumić K, Krečak F, Holik H, Duraković N
Acta Clin Belg 2021 Feb;76(1):32-39. Epub 2019 Aug 27 doi: 10.1080/17843286.2019.1659467. PMID: 31455178
Treating early-stage myelofibrosis.
Palandri F, Sabattini E, Maffioli M
Ann Hematol 2019 Feb;98(2):241-253. Epub 2018 Oct 20 doi: 10.1007/s00277-018-3526-z. PMID: 30343328
Inflammation and myeloproliferative neoplasms.
Lussana F, Rambaldi A
J Autoimmun 2017 Dec;85:58-63. Epub 2017 Jun 30 doi: 10.1016/j.jaut.2017.06.010. PMID: 28669446
Emerging treatments for classical myeloproliferative neoplasms.
Vannucchi AM, Harrison CN
Blood 2017 Feb 9;129(6):693-703. Epub 2016 Dec 27 doi: 10.1182/blood-2016-10-695965. PMID: 28028027

Diagnosis

Momelotinib long-term safety and survival in myelofibrosis: integrated analysis of phase 3 randomized controlled trials.
Verstovsek S, Mesa R, Gupta V, Lavie D, Dubruille V, Cambier N, Platzbecker U, Hus M, Xicoy B, Oh ST, Kiladjian JJ, Vannucchi AM, Gerds A, Egyed M, Mayer J, Sacha T, Kawashima J, Morris M, Huang M, Harrison C
Blood Adv 2023 Jul 25;7(14):3582-3591. doi: 10.1182/bloodadvances.2022009311. PMID: 37042865Free PMC Article
JAK2 V617F allele burden in polycythemia vera: burden of proof.
Moliterno AR, Kaizer H, Reeves BN
Blood 2023 Apr 20;141(16):1934-1942. doi: 10.1182/blood.2022017697. PMID: 36745865Free PMC Article
Novel therapeutics and targets in myelofibrosis.
Waksal JA, Harrison CN, Mascarenhas JO
Leuk Lymphoma 2022 May;63(5):1020-1033. Epub 2021 Dec 2 doi: 10.1080/10428194.2021.2010068. PMID: 34852713
Treating early-stage myelofibrosis.
Palandri F, Sabattini E, Maffioli M
Ann Hematol 2019 Feb;98(2):241-253. Epub 2018 Oct 20 doi: 10.1007/s00277-018-3526-z. PMID: 30343328
Inflammation and myeloproliferative neoplasms.
Lussana F, Rambaldi A
J Autoimmun 2017 Dec;85:58-63. Epub 2017 Jun 30 doi: 10.1016/j.jaut.2017.06.010. PMID: 28669446

Therapy

Momelotinib long-term safety and survival in myelofibrosis: integrated analysis of phase 3 randomized controlled trials.
Verstovsek S, Mesa R, Gupta V, Lavie D, Dubruille V, Cambier N, Platzbecker U, Hus M, Xicoy B, Oh ST, Kiladjian JJ, Vannucchi AM, Gerds A, Egyed M, Mayer J, Sacha T, Kawashima J, Morris M, Huang M, Harrison C
Blood Adv 2023 Jul 25;7(14):3582-3591. doi: 10.1182/bloodadvances.2022009311. PMID: 37042865Free PMC Article
Phase III MANIFEST-2: pelabresib + ruxolitinib vs placebo + ruxolitinib in JAK inhibitor treatment-naive myelofibrosis.
Harrison CN, Gupta VK, Gerds AT, Rampal R, Verstovsek S, Talpaz M, Kiladjian JJ, Mesa R, Kuykendall AT, Vannucchi AM, Palandri F, Grosicki S, Devos T, Jourdan E, Wondergem MJ, Al-Ali HK, Buxhofer-Ausch V, Alvarez-Larrán A, Patriarca A, Kremyanskaya M, Mead AJ, Akhani S, Sheikine Y, Colak G, Mascarenhas J
Future Oncol 2022 Sep;18(27):2987-2997. Epub 2022 Aug 11 doi: 10.2217/fon-2022-0484. PMID: 35950489
Novel therapeutics and targets in myelofibrosis.
Waksal JA, Harrison CN, Mascarenhas JO
Leuk Lymphoma 2022 May;63(5):1020-1033. Epub 2021 Dec 2 doi: 10.1080/10428194.2021.2010068. PMID: 34852713
Investigational non-JAK inhibitors for chronic phase myelofibrosis.
Bankar A, Gupta V
Expert Opin Investig Drugs 2020 May;29(5):461-474. Epub 2020 Apr 29 doi: 10.1080/13543784.2020.1751121. PMID: 32245330
Treating early-stage myelofibrosis.
Palandri F, Sabattini E, Maffioli M
Ann Hematol 2019 Feb;98(2):241-253. Epub 2018 Oct 20 doi: 10.1007/s00277-018-3526-z. PMID: 30343328

Prognosis

JAK2 V617F allele burden in polycythemia vera: burden of proof.
Moliterno AR, Kaizer H, Reeves BN
Blood 2023 Apr 20;141(16):1934-1942. doi: 10.1182/blood.2022017697. PMID: 36745865Free PMC Article
Molecular Pathogenesis of Myeloproliferative Neoplasms.
Rolles B, Mullally A
Curr Hematol Malig Rep 2022 Dec;17(6):319-329. Epub 2022 Nov 7 doi: 10.1007/s11899-022-00685-1. PMID: 36336766
Circulating YKL-40 in Philadelphia-negative myeloproliferative neoplasms.
Krečak I, Gverić-Krečak V, Lapić I, Rončević P, Gulin J, Fumić K, Krečak F, Holik H, Duraković N
Acta Clin Belg 2021 Feb;76(1):32-39. Epub 2019 Aug 27 doi: 10.1080/17843286.2019.1659467. PMID: 31455178
Treating early-stage myelofibrosis.
Palandri F, Sabattini E, Maffioli M
Ann Hematol 2019 Feb;98(2):241-253. Epub 2018 Oct 20 doi: 10.1007/s00277-018-3526-z. PMID: 30343328
Inflammation and myeloproliferative neoplasms.
Lussana F, Rambaldi A
J Autoimmun 2017 Dec;85:58-63. Epub 2017 Jun 30 doi: 10.1016/j.jaut.2017.06.010. PMID: 28669446

Clinical prediction guides

A Phase Ib Trial of AVID200, a TGFβ 1/3 Trap, in Patients with Myelofibrosis.
Mascarenhas J, Migliaccio AR, Kosiorek H, Bhave R, Palmer J, Kuykendall A, Mesa R, Rampal RK, Gerds AT, Yacoub A, Pettit K, Talpaz M, Komrokji R, Kremyanskaya M, Gonzalez A, Fabris F, Johnson K, Dougherty M, McGovern E, Arango Ossa J, Domenico D, Farnoud N, Weinberg RS, Kong A, Najfeld V, Vannucchi AM, Arciprete F, Zingariello M, Falchi M, Salama ME, Mead-Harvey C, Dueck A, Varricchio L, Hoffman R
Clin Cancer Res 2023 Sep 15;29(18):3622-3632. doi: 10.1158/1078-0432.CCR-23-0276. PMID: 37439808Free PMC Article
PD-1 inhibition in advanced myeloproliferative neoplasms.
Hobbs G, Cimen Bozkus C, Moshier E, Dougherty M, Bar-Natan M, Sandy L, Johnson K, Foster JE, Som T, Macrae M, Marble H, Salama M, El Jamal SM, Zubizarreta N, Wadleigh M, Stone R, Bhardwaj N, Iancu-Rubin C, Mascarenhas J
Blood Adv 2021 Dec 14;5(23):5086-5097. doi: 10.1182/bloodadvances.2021005491. PMID: 34581778Free PMC Article
Circulating YKL-40 in Philadelphia-negative myeloproliferative neoplasms.
Krečak I, Gverić-Krečak V, Lapić I, Rončević P, Gulin J, Fumić K, Krečak F, Holik H, Duraković N
Acta Clin Belg 2021 Feb;76(1):32-39. Epub 2019 Aug 27 doi: 10.1080/17843286.2019.1659467. PMID: 31455178
Treating early-stage myelofibrosis.
Palandri F, Sabattini E, Maffioli M
Ann Hematol 2019 Feb;98(2):241-253. Epub 2018 Oct 20 doi: 10.1007/s00277-018-3526-z. PMID: 30343328
Ruxolitinib: a review of its use in patients with myelofibrosis.
Plosker GL
Drugs 2015 Feb;75(3):297-308. doi: 10.1007/s40265-015-0351-8. PMID: 25601187

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