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Overt Primary Myelofibrosis

MedGen UID:
308982
•Concept ID:
C1516552
•
Neoplastic Process
Synonyms: Chronic Idiopathic Myelofibrosis, Fibrotic Stage; Primary Myelofibrosis, Fibrotic Stage; Primary Myelofibrosis, Overt Fibrotic Stage

Definition

Primary myelofibrosis characterized by reticulin or collagen fibrosis in the bone marrow. The bone marrow is usually normocellular or hypocellular. Myeloblasts account for less than 10% of the bone marrow cells. Atypical megakaryocytes are present. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Diagnosis and treatment of MPN in real life: exploratory and retrospective chart review including 960 MPN patients diagnosed with ET or MF in Germany.
Schmidt A, Bernhardt C, Bürkle D, Fries S, Hannig CV, Jentsch-Ullrich K, Josting A, Kreher S, Reiser M, Steinmetz HT, Tesch H, Terner S, Schulte A, Crodel CC, Palandri F, Heidel FH
J Cancer Res Clin Oncol 2023 Aug;149(10):7197-7206. Epub 2023 Mar 8 doi: 10.1007/s00432-023-04669-3. PMID: 36884118Free PMC Article
Prefibrotic myelofibrosis: treatment algorithm 2018.
Finazzi G, Vannucchi AM, Barbui T
Blood Cancer J 2018 Nov 7;8(11):104. doi: 10.1038/s41408-018-0142-z. PMID: 30405096Free PMC Article
Diagnosis and management of prefibrotic myelofibrosis.
Rumi E, Sant'Antonio E, Boveri E, Pietra D, Cavalloni C, Roncoroni E, Astori C, Arcaini L; Associazione Italiana per la Ricerca sul Cancro Gruppo Italiano Malattie Mieloproliferative Investigators
Expert Rev Hematol 2018 Jul;11(7):537-545. Epub 2018 Jun 22 doi: 10.1080/17474086.2018.1484280. PMID: 29862872

Recent clinical studies

Etiology

Challenges of diagnosing and managing pre-fibrotic myelofibrosis: A case-based and practical approach.
Rampotas A, Hargreaves R, McLornan DP
Best Pract Res Clin Haematol 2022 Jun;35(2):101378. Epub 2022 Aug 24 doi: 10.1016/j.beha.2022.101378. PMID: 36333067
Towards a Personalized Definition of Prognosis in Philadelphia-Negative Myeloproliferative Neoplasms.
Mora B, Passamonti F
Curr Hematol Malig Rep 2022 Oct;17(5):127-139. Epub 2022 Sep 1 doi: 10.1007/s11899-022-00672-6. PMID: 36048275Free PMC Article
Impact of 2016 WHO diagnosis of early and overt primary myelofibrosis on presentation and outcome of 232 patients treated with ruxolitinib.
Palandri F, Palumbo GA, Abruzzese E, Iurlo A, Polverelli N, Elli E, Bonifacio M, Bergamaschi M, Martino B, Tiribelli M, Benevolo G, Tieghi A, Sgherza N, Isidori A, Binotto G, Crugnola M, Heidel F, Cavazzini F, Bosi C, Auteri G, Cattaneo D, Foà R, Lemoli RM, Cuneo A, Krampera M, Bartoletti D, Cavo M, Vianelli N, Breccia M, Latagliata R
Hematol Oncol 2019 Oct;37(4):418-423. Epub 2019 Jun 7 doi: 10.1002/hon.2619. PMID: 30985017
Presentation and outcome of patients with 2016 WHO diagnosis of prefibrotic and overt primary myelofibrosis.
Guglielmelli P, Pacilli A, Rotunno G, Rumi E, Rosti V, Delaini F, Maffioli M, Fanelli T, Pancrazzi A, Pietra D, Salmoiraghi S, Mannarelli C, Franci A, Paoli C, Rambaldi A, Passamonti F, Barosi G, Barbui T, Cazzola M, Vannucchi AM; AGIMM Group
Blood 2017 Jun 15;129(24):3227-3236. Epub 2017 Mar 28 doi: 10.1182/blood-2017-01-761999. PMID: 28351937

Diagnosis

Challenges of diagnosing and managing pre-fibrotic myelofibrosis: A case-based and practical approach.
Rampotas A, Hargreaves R, McLornan DP
Best Pract Res Clin Haematol 2022 Jun;35(2):101378. Epub 2022 Aug 24 doi: 10.1016/j.beha.2022.101378. PMID: 36333067
Towards a Personalized Definition of Prognosis in Philadelphia-Negative Myeloproliferative Neoplasms.
Mora B, Passamonti F
Curr Hematol Malig Rep 2022 Oct;17(5):127-139. Epub 2022 Sep 1 doi: 10.1007/s11899-022-00672-6. PMID: 36048275Free PMC Article
GATA1 Expression in BCR/ABL1-negative Myeloproliferative Neoplasms.
Yang N, Park S, Cho MS, Lee M, Hong KS, Mun YC, Seong CM, Huh HJ, Huh J
Ann Lab Med 2018 Jul;38(4):296-305. doi: 10.3343/alm.2018.38.4.296. PMID: 29611379Free PMC Article
Presentation and outcome of patients with 2016 WHO diagnosis of prefibrotic and overt primary myelofibrosis.
Guglielmelli P, Pacilli A, Rotunno G, Rumi E, Rosti V, Delaini F, Maffioli M, Fanelli T, Pancrazzi A, Pietra D, Salmoiraghi S, Mannarelli C, Franci A, Paoli C, Rambaldi A, Passamonti F, Barosi G, Barbui T, Cazzola M, Vannucchi AM; AGIMM Group
Blood 2017 Jun 15;129(24):3227-3236. Epub 2017 Mar 28 doi: 10.1182/blood-2017-01-761999. PMID: 28351937
The 2016 revision of WHO classification of myeloproliferative neoplasms: Clinical and molecular advances.
Barbui T, Thiele J, Gisslinger H, Finazzi G, Vannucchi AM, Tefferi A
Blood Rev 2016 Nov;30(6):453-459. Epub 2016 Jun 11 doi: 10.1016/j.blre.2016.06.001. PMID: 27341755

Therapy

Impact of 2016 WHO diagnosis of early and overt primary myelofibrosis on presentation and outcome of 232 patients treated with ruxolitinib.
Palandri F, Palumbo GA, Abruzzese E, Iurlo A, Polverelli N, Elli E, Bonifacio M, Bergamaschi M, Martino B, Tiribelli M, Benevolo G, Tieghi A, Sgherza N, Isidori A, Binotto G, Crugnola M, Heidel F, Cavazzini F, Bosi C, Auteri G, Cattaneo D, Foà R, Lemoli RM, Cuneo A, Krampera M, Bartoletti D, Cavo M, Vianelli N, Breccia M, Latagliata R
Hematol Oncol 2019 Oct;37(4):418-423. Epub 2019 Jun 7 doi: 10.1002/hon.2619. PMID: 30985017
Presentation and outcome of patients with 2016 WHO diagnosis of prefibrotic and overt primary myelofibrosis.
Guglielmelli P, Pacilli A, Rotunno G, Rumi E, Rosti V, Delaini F, Maffioli M, Fanelli T, Pancrazzi A, Pietra D, Salmoiraghi S, Mannarelli C, Franci A, Paoli C, Rambaldi A, Passamonti F, Barosi G, Barbui T, Cazzola M, Vannucchi AM; AGIMM Group
Blood 2017 Jun 15;129(24):3227-3236. Epub 2017 Mar 28 doi: 10.1182/blood-2017-01-761999. PMID: 28351937

Prognosis

Challenges of diagnosing and managing pre-fibrotic myelofibrosis: A case-based and practical approach.
Rampotas A, Hargreaves R, McLornan DP
Best Pract Res Clin Haematol 2022 Jun;35(2):101378. Epub 2022 Aug 24 doi: 10.1016/j.beha.2022.101378. PMID: 36333067
Towards a Personalized Definition of Prognosis in Philadelphia-Negative Myeloproliferative Neoplasms.
Mora B, Passamonti F
Curr Hematol Malig Rep 2022 Oct;17(5):127-139. Epub 2022 Sep 1 doi: 10.1007/s11899-022-00672-6. PMID: 36048275Free PMC Article
GATA1 Expression in BCR/ABL1-negative Myeloproliferative Neoplasms.
Yang N, Park S, Cho MS, Lee M, Hong KS, Mun YC, Seong CM, Huh HJ, Huh J
Ann Lab Med 2018 Jul;38(4):296-305. doi: 10.3343/alm.2018.38.4.296. PMID: 29611379Free PMC Article
Presentation and outcome of patients with 2016 WHO diagnosis of prefibrotic and overt primary myelofibrosis.
Guglielmelli P, Pacilli A, Rotunno G, Rumi E, Rosti V, Delaini F, Maffioli M, Fanelli T, Pancrazzi A, Pietra D, Salmoiraghi S, Mannarelli C, Franci A, Paoli C, Rambaldi A, Passamonti F, Barosi G, Barbui T, Cazzola M, Vannucchi AM; AGIMM Group
Blood 2017 Jun 15;129(24):3227-3236. Epub 2017 Mar 28 doi: 10.1182/blood-2017-01-761999. PMID: 28351937
The 2016 revision of WHO classification of myeloproliferative neoplasms: Clinical and molecular advances.
Barbui T, Thiele J, Gisslinger H, Finazzi G, Vannucchi AM, Tefferi A
Blood Rev 2016 Nov;30(6):453-459. Epub 2016 Jun 11 doi: 10.1016/j.blre.2016.06.001. PMID: 27341755

Clinical prediction guides

Towards a Personalized Definition of Prognosis in Philadelphia-Negative Myeloproliferative Neoplasms.
Mora B, Passamonti F
Curr Hematol Malig Rep 2022 Oct;17(5):127-139. Epub 2022 Sep 1 doi: 10.1007/s11899-022-00672-6. PMID: 36048275Free PMC Article
Impact of 2016 WHO diagnosis of early and overt primary myelofibrosis on presentation and outcome of 232 patients treated with ruxolitinib.
Palandri F, Palumbo GA, Abruzzese E, Iurlo A, Polverelli N, Elli E, Bonifacio M, Bergamaschi M, Martino B, Tiribelli M, Benevolo G, Tieghi A, Sgherza N, Isidori A, Binotto G, Crugnola M, Heidel F, Cavazzini F, Bosi C, Auteri G, Cattaneo D, Foà R, Lemoli RM, Cuneo A, Krampera M, Bartoletti D, Cavo M, Vianelli N, Breccia M, Latagliata R
Hematol Oncol 2019 Oct;37(4):418-423. Epub 2019 Jun 7 doi: 10.1002/hon.2619. PMID: 30985017
Presentation and outcome of patients with 2016 WHO diagnosis of prefibrotic and overt primary myelofibrosis.
Guglielmelli P, Pacilli A, Rotunno G, Rumi E, Rosti V, Delaini F, Maffioli M, Fanelli T, Pancrazzi A, Pietra D, Salmoiraghi S, Mannarelli C, Franci A, Paoli C, Rambaldi A, Passamonti F, Barosi G, Barbui T, Cazzola M, Vannucchi AM; AGIMM Group
Blood 2017 Jun 15;129(24):3227-3236. Epub 2017 Mar 28 doi: 10.1182/blood-2017-01-761999. PMID: 28351937
The 2016 revision of WHO classification of myeloproliferative neoplasms: Clinical and molecular advances.
Barbui T, Thiele J, Gisslinger H, Finazzi G, Vannucchi AM, Tefferi A
Blood Rev 2016 Nov;30(6):453-459. Epub 2016 Jun 11 doi: 10.1016/j.blre.2016.06.001. PMID: 27341755

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