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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

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
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
Michiels JJ, Thiele J
Int J Hematol 2002 Aug;76(2):133-45. doi: 10.1007/BF02982575. PMID: 12215011

Recent clinical studies

Etiology

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
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
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
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

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
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
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
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
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

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
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

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
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
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
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
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

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
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
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
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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