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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

2024 update on allogeneic hematopoietic stem cell transplant for myelofibrosis: A review of current data and applications on risk stratification and management.
Ali H, Bacigalupo A
Am J Hematol 2024 May;99(5):938-945. Epub 2024 Mar 7 doi: 10.1002/ajh.27274. PMID: 38450790
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

2024 update on allogeneic hematopoietic stem cell transplant for myelofibrosis: A review of current data and applications on risk stratification and management.
Ali H, Bacigalupo A
Am J Hematol 2024 May;99(5):938-945. Epub 2024 Mar 7 doi: 10.1002/ajh.27274. PMID: 38450790
Meaningful Symptomatic Change in Patients With Myelofibrosis From the SIMPLIFY Studies.
Hudgens S, Verstovsek S, Floden L, Harrison CN, Palmer J, Gupta V, McLornan D, McMullin MF, Kiladjian JJ, Foltz L, Platzbecker U, Fox ML, Mead AJ, Ross DM, Oh ST, Perkins AA, Leahy MF, Deheshi S, Donahue R, Klencke BJ, Mesa RA
Value Health 2024 May;27(5):607-613. Epub 2024 Feb 2 doi: 10.1016/j.jval.2024.01.014. PMID: 38311180
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
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

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
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
Cooperation between pathologists and clinicians allows a better diagnosis of Philadelphia chromosome-negative myeloproliferative neoplasms.
Gianelli U, Iurlo A, Cattaneo D, Lambertenghi-Deliliers G
Expert Rev Hematol 2014 Apr;7(2):255-64. Epub 2014 Feb 13 doi: 10.1586/17474086.2014.876898. PMID: 24524231
Mast cells and myelofibrosis.
Udoji WC, Razavi SA
Am J Clin Pathol 1975 Feb;63(2):203-9. doi: 10.1093/ajcp/63.2.203. PMID: 1115032

Therapy

2024 update on allogeneic hematopoietic stem cell transplant for myelofibrosis: A review of current data and applications on risk stratification and management.
Ali H, Bacigalupo A
Am J Hematol 2024 May;99(5):938-945. Epub 2024 Mar 7 doi: 10.1002/ajh.27274. PMID: 38450790
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
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
Cooperation between pathologists and clinicians allows a better diagnosis of Philadelphia chromosome-negative myeloproliferative neoplasms.
Gianelli U, Iurlo A, Cattaneo D, Lambertenghi-Deliliers G
Expert Rev Hematol 2014 Apr;7(2):255-64. Epub 2014 Feb 13 doi: 10.1586/17474086.2014.876898. PMID: 24524231

Prognosis

2024 update on allogeneic hematopoietic stem cell transplant for myelofibrosis: A review of current data and applications on risk stratification and management.
Ali H, Bacigalupo A
Am J Hematol 2024 May;99(5):938-945. Epub 2024 Mar 7 doi: 10.1002/ajh.27274. PMID: 38450790
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
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

Prognostic and Predictive Models in Myelofibrosis.
Mora B, Bucelli C, Cattaneo D, Bellani V, Versino F, Barbullushi K, Fracchiolla N, Iurlo A, Passamonti F
Curr Hematol Malig Rep 2024 Oct;19(5):223-235. Epub 2024 Aug 24 doi: 10.1007/s11899-024-00739-6. PMID: 39179882Free PMC Article
2024 update on allogeneic hematopoietic stem cell transplant for myelofibrosis: A review of current data and applications on risk stratification and management.
Ali H, Bacigalupo A
Am J Hematol 2024 May;99(5):938-945. Epub 2024 Mar 7 doi: 10.1002/ajh.27274. PMID: 38450790
Meaningful Symptomatic Change in Patients With Myelofibrosis From the SIMPLIFY Studies.
Hudgens S, Verstovsek S, Floden L, Harrison CN, Palmer J, Gupta V, McLornan D, McMullin MF, Kiladjian JJ, Foltz L, Platzbecker U, Fox ML, Mead AJ, Ross DM, Oh ST, Perkins AA, Leahy MF, Deheshi S, Donahue R, Klencke BJ, Mesa RA
Value Health 2024 May;27(5):607-613. Epub 2024 Feb 2 doi: 10.1016/j.jval.2024.01.014. PMID: 38311180
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
A phase-2 trial of low-dose pomalidomide in myelofibrosis.
Begna KH, Mesa RA, Pardanani A, Hogan WJ, Litzow MR, McClure RF, Tefferi A
Leukemia 2011 Feb;25(2):301-4. Epub 2010 Nov 5 doi: 10.1038/leu.2010.254. PMID: 21052089

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