Skip to main content
SpringerLink
Log in

Risk Stratification in PMF

  • Chapter
  • First Online:
Myeloproliferative Neoplasms

Part of the book series: Hematologic Malignancies ((HEMATOLOGIC))

  • 998 Accesses

Abstract

Primary myelofibrosis (PMF) is a Philadelphia-negative myeloproliferative neoplasm (MPN) whose diagnostic criteria have been recently updated (Tefferi et al. 2007). Among MPNs, PMF has the most heterogeneous clinical presentation, which may encompass anemia, splenomegaly, leukocytosis or leukopenia, thrombocytosis or thrombocytopenia, and constitutional symptoms. Median survival in PMF is estimated at 6 years, but it can range from few months to many years (Cervantes et al. 2009; Passamonti et al. 2010a; Gangat et al. 2011; Tam et al. 2009).

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  • Al-Assar O, Ul-Hassan A, Brown R, Wilson GA, Hammond DW, Reilly JT (2005) Gains on 9p are common genomic aberrations in idiopathic myelofibrosis: a comparative genomic hybridization study. Br J Haematol 129:66–71

    Article  PubMed  CAS  Google Scholar 

  • Alchalby H, Badbaran A, Zabelina T et al (2010) Impact of JAK2V617F-mutation status, allele burden and clearance after allogeneic stem cell transplantation for myelofibrosis. Blood 116(18):3572–3581

    Article  PubMed  CAS  Google Scholar 

  • Alchalby H, Lioznov M, Fritzsche-Friedland U et al (2011) Circulating CD34(+) cells as prognostic and follow-up marker in patients with myelofibrosis undergoing allo-SCT. Bone Marrow Transplant. doi:10.1038/bmt.2011.17

  • Andrieux J, Demory JL, Caulier MT et al (2003) Karyotypic abnormalities in myelofibrosis following polycythemia vera. Cancer Genet Cytogenet 140:118–123

    Article  PubMed  CAS  Google Scholar 

  • Arora B, Sirhan S, Hoyer JD, Mesa RA, Tefferi A (2005) Peripheral blood CD34 count in myelofibrosis with myeloid metaplasia: a prospective evaluation of prognostic value in 94 patients. Br J Haematol 128:42–48

    Article  PubMed  Google Scholar 

  • Barbui T, Barosi G, Birgegard G et al (2011) Philadelphia-negative classical myeloproliferative neoplasms: critical concepts and management recommendations from European LeukemiaNet. J Clin Oncol 29:761–770

    Article  PubMed  Google Scholar 

  • Barosi G, Berzuini C, Liberato LN, Costa A, Polino G, Ascari E (1988) A prognostic classification of myelofibrosis with myeloid metaplasia. Br J Haematol 70:397–401

    Article  PubMed  CAS  Google Scholar 

  • Barosi G, Viarengo G, Pecci A et al (2001) Diagnostic and clinical relevance of the number of circulating CD34(+) cells in myelofibrosis with myeloid metaplasia. Blood 98:3249–3255

    Article  PubMed  CAS  Google Scholar 

  • Barosi G, Bergamaschi G, Marchetti M et al (2007) JAK2 V617F mutational status predicts progression to large splenomegaly and leukemic transformation in primary myelofibrosis. Blood 110:4030–4036

    Article  PubMed  CAS  Google Scholar 

  • Campbell PJ, Griesshammer M, Dohner K et al (2006) V617F mutation in JAK2 is associated with poorer survival in idiopathic myelofibrosis. Blood 107:2098–2100

    Article  PubMed  CAS  Google Scholar 

  • Caramazza D, Hussein K, Siragusa S et al (2010) Chromosome 1 abnormalities in myeloid malignancies: a literature survey and karyotype-phenotype associations. Eur J Haematol 84:191–200

    Article  PubMed  CAS  Google Scholar 

  • Caramazza D, Begna KH, Gangat N et al (2011) Refined cytogenetic-risk categorization for overall and leukemia-free survival in primary myelofibrosis: a single center study of 433 patients. Leukemia 25:82–88

    Article  PubMed  CAS  Google Scholar 

  • Cervantes F, Pereira A, Esteve J et al (1997) Identification of ‘short-lived’ and ‘long-lived’ patients at presentation of idiopathic myelofibrosis. Br J Haematol 97:635–640

    Article  PubMed  CAS  Google Scholar 

  • Cervantes F, Dupriez B, Pereira A et al (2009) New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment. Blood 113:2895–2901

    Article  PubMed  CAS  Google Scholar 

  • Chee CE, Steensma DP, Wu W, Hanson CA, Tefferi A (2008) Neither serum ferritin nor the number of red blood cell transfusions affect overall survival in refractory anemia with ringed sideroblasts. Am J Hematol 83:611–613

    Article  PubMed  CAS  Google Scholar 

  • Chelloul N, Briere J, Laval-Jeantet M, Najean Y, Vorhauer W, Jacquillat C (1976) Prognosis of myeloid metaplasia with myelofibrosis. Biomedicine 24:272–280

    PubMed  CAS  Google Scholar 

  • Dingli D, Grand FH, Mahaffey V et al (2005) Der(6)t(1;6)(q21–23;p21.3): a specific cytogenetic abnormality in myelofibrosis with myeloid metaplasia. Br J Haematol 130:229–232

    Article  PubMed  CAS  Google Scholar 

  • Dupriez B, Morel P, Demory JL et al (1996) Prognostic factors in agnogenic myeloid metaplasia: a report on 195 cases with a new scoring system. Blood 88:1013–1018

    PubMed  CAS  Google Scholar 

  • Elena C, Passamonti F, Rumi E et al (2011) Red blood cell transfusion-dependency implies a poor survival in primary myelofibrosis irrespective of IPSS and DIPSS. Haematologica 96:167–170

    Article  PubMed  Google Scholar 

  • Gale RP, Barosi G, Barbui T et al (2011) What are RBC-transfusion-dependence and -independence? Leuk Res 35:8–11

    Article  PubMed  CAS  Google Scholar 

  • Gangat N, Caramazza D, Vaidya R et al (2011) DIPSS plus: a refined Dynamic International Prognostic Scoring System for primary myelofibrosis that incorporates prognostic information from karyotype, platelet count, and transfusion status. J Clin Oncol 29:392–397

    Article  PubMed  Google Scholar 

  • Guglielmelli P, Pancrazzi A, Bergamaschi G et al (2007) Anaemia characterises patients with myelofibrosis harbouring Mpl mutation. Br J Haematol 137:244–247

    Article  PubMed  CAS  Google Scholar 

  • Guglielmelli P, Barosi G, Specchia G et al (2009) Identification of patients with poorer survival in primary myelofibrosis based on the burden of JAK2V617F mutated allele. Blood 114:1477–1483

    Article  PubMed  CAS  Google Scholar 

  • Helias C, Struski S, Gervais C et al (2008) Polycythemia vera transforming to acute myeloid leukemia and complex abnormalities including 9p homogeneously staining region with amplification of MLLT3, JMJD2C, JAK2, and SMARCA2. Cancer Genet Cytogenet 180:51–55

    Article  PubMed  CAS  Google Scholar 

  • Hidaka T, Shide K, Shimoda H et al (2009) The impact of cytogenetic abnormalities on the prognosis of primary myelofibrosis: a prospective survey of 202 cases in Japan. Eur J Haematol 83:328–333

    Article  PubMed  Google Scholar 

  • Hussein K, Huang J, Lasho T et al (2009a) Karyotype complements the International Prognostic Scoring System for primary myelofibrosis. Eur J Haematol 82:255–259

    Article  PubMed  Google Scholar 

  • Hussein K, Van Dyke DL, Tefferi A (2009b) Conventional cytogenetics in myelofibrosis: literature review and discussion. Eur J Haematol 82:329–338

    Article  PubMed  Google Scholar 

  • Hussein K, Pardanani AD, Van Dyke DL, Hanson CA, Tefferi A (2010) International Prognostic Scoring System-independent cytogenetic risk categorization in primary myelofibrosis. Blood 115:496–499

    Article  PubMed  CAS  Google Scholar 

  • Malcovati L, Porta MG, Pascutto C et al (2005) Prognostic factors and life expectancy in myelodysplastic syndromes classified according to WHO criteria: a basis for clinical decision making. J Clin Oncol 23:7594–7603

    Article  PubMed  Google Scholar 

  • Mesa RA, Hanson CA, Rajkumar SV, Schroeder G, Tefferi A (2000) Evaluation and clinical correlations of bone marrow angiogenesis in myelofibrosis with myeloid metaplasia. Blood 96:3374–3380

    PubMed  CAS  Google Scholar 

  • Mesa RA, Powell H, Lasho T, DeWald GW, McClure R, Tefferi A (2006) A longitudinal study of the JAK2(V617F) mutation in myelofibrosis with myeloid metaplasia: analysis at two time points. Haematologica 91:415–416

    PubMed  CAS  Google Scholar 

  • Morel P, Duhamel A, Hivert B, Stalniekiewicz L, Demory JL, Dupriez B (2010) Identification during the follow-up of time-dependent prognostic factors for the competing risks of death and blast phase in primary myelofibrosis: a study of 172 patients. Blood 115:4350–4355

    Article  PubMed  CAS  Google Scholar 

  • Pardanani A, Vannucchi AM, Passamonti F, Cervantes F, Barbui T, Tefferi A (2011) JAK inhibitor therapy for myelofibrosis: critical assessment of value and limitations. Leukemia 25:218–225

    Article  PubMed  CAS  Google Scholar 

  • Passamonti F, Cervantes F, Vannucchi AM et al (2010a) A dynamic prognostic model to predict survival in primary myelofibrosis: a study by the IWG-MRT (International Working Group for Myeloproliferative Neoplasms Research and Treatment). Blood 115:1703–1708

    Article  PubMed  CAS  Google Scholar 

  • Passamonti F, Cervantes F, Vannucchi AM et al (2010b) Dynamic International Prognostic Scoring System (DIPSS) predicts progression to acute myeloid leukemia in primary myelofibrosis. Blood 116:2857–2858

    Article  PubMed  CAS  Google Scholar 

  • Patnaik MM, Caramazza D, Gangat N, Hanson CA, Pardanani A, Tefferi A (2010) Age and platelet count are IPSS-independent prognostic factors in young patients with primary myelofibrosis and complement IPSS in predicting very long or very short survival. Eur J Haematol 84:105–108

    Article  PubMed  Google Scholar 

  • Pereira A, Bruguera M, Cervantes F, Rozman C (1988) Liver involvement at diagnosis of primary myelofibrosis: a clinicopathological study of twenty-two cases. Eur J Haematol 40:355–361

    Article  PubMed  CAS  Google Scholar 

  • Pikman Y, Lee BH, Mercher T et al (2006) MPLW515L is a novel somatic activating mutation in myelofibrosis with myeloid metaplasia. PLoS Med 3:e270

    Article  PubMed  Google Scholar 

  • Reilly JT, Snowden JA, Spearing RL et al (1997) Cytogenetic abnormalities and their prognostic significance in idiopathic myelofibrosis: a study of 106 cases. Br J Haematol 98:96–102

    Article  PubMed  CAS  Google Scholar 

  • Rumi E, Passamonti F, Bernasconi P et al (2010) Validation of cytogenetic-based risk stratification in primary myelofibrosis. Blood 115:2719–2720

    Article  PubMed  CAS  Google Scholar 

  • Rupoli S, Da Lio L, Sisti S et al (1994) Primary myelofibrosis: a detailed statistical analysis of the clinicopathological variables influencing survival. Ann Hematol 68:205–212

    Article  PubMed  CAS  Google Scholar 

  • Sinclair EJ, Forrest EC, Reilly JT, Watmore AE, Potter AM (2001) Fluorescence in situ hybridization analysis of 25 cases of idiopathic myelofibrosis and two cases of secondary myelofibrosis: monoallelic loss of RB1, D13S319 and D13S25 loci associated with cytogenetic deletion and translocation involving 13q14. Br J Haematol 113:365–368

    Article  PubMed  CAS  Google Scholar 

  • Tam CS, Abruzzo LV, Lin KI et al (2009) The role of cytogenetic abnormalities as a prognostic marker in primary myelofibrosis: applicability at the time of diagnosis and later during disease course. Blood 113:4171–4178

    Article  PubMed  CAS  Google Scholar 

  • Tefferi A, Mesa RA, Schroeder G, Hanson CA, Li CY, Dewald GW (2001a) Cytogenetic findings and their clinical relevance in myelofibrosis with myeloid metaplasia. Br J Haematol 113:763–771

    Article  PubMed  CAS  Google Scholar 

  • Tefferi A, Meyer RG, Wyatt WA, Dewald GW (2001b) Comparison of peripheral blood interphase cytogenetics with bone marrow karyotype analysis in myelofibrosis with myeloid metaplasia. Br J Haematol 115:316–319

    Article  PubMed  CAS  Google Scholar 

  • Tefferi A, Thiele J, Orazi A et al (2007) Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel. Blood 110:1092–1097

    Article  PubMed  CAS  Google Scholar 

  • Tefferi A, Lasho TL, Huang J et al (2008) Low JAK2V617F allele burden in primary myelofibrosis, compared to either a higher allele burden or unmutated status, is associated with inferior overall and leukemia-free survival. Leukemia 22:756–761

    Article  PubMed  CAS  Google Scholar 

  • Tefferi A, Mesa RA, Pardanani A et al (2009a) Red blood cell transfusion need at diagnosis adversely affects survival in primary myelofibrosis-increased serum ferritin or transfusion load does not. Am J Hematol 84:265–267

    Article  PubMed  CAS  Google Scholar 

  • Tefferi A, Pardanani A, Lim KH et al (2009b) TET2 mutations and their clinical correlates in polycythemia vera, essential thrombocythemia and myelofibrosis. Leuk­emia 23(5):905–911

    Article  PubMed  CAS  Google Scholar 

  • Tefferi A, Siragusa S, Hussein K et al (2010a) Transfusion-dependency at presentation and its acquisition in the first year of diagnosis are both equally detrimental for survival in primary myelofibrosis–prognostic relevance is independent of IPSS or karyotype. Am J Hematol 85:14–17

    Article  PubMed  Google Scholar 

  • Tefferi A, Lasho TL, Patnaik MM et al (2010b) JAK2 germline genetic variation affects disease susceptibility in primary myelofibrosis regardless of V617F mutational status: nullizygosity for the JAK2 46/1 haplotype is associated with inferior survival. Leukemia 24:105–109

    Article  PubMed  CAS  Google Scholar 

  • Tefferi A, Vaidya R, Caramazza D, Finke C, Lasho T, Pardanani A (2011) Circulating interleukin (IL)-8, IL-2R, IL-12, and IL-15 levels are independently prognostic in primary myelofibrosis: a comprehensive cytokine profiling study. J Clin Oncol 29:1356–1363

    Article  PubMed  CAS  Google Scholar 

  • Vaidya R, Caramazza D, Begna KH et al (2011) Monosomal karyotype in primary myelofibrosis is detrimental to both overall and leukemia-free survival. Blood 117(21):5612–5615

    Article  PubMed  CAS  Google Scholar 

  • Verstovsek S, Kantarjian H, Mesa RA et al (2010) Safety and efficacy of INCB018424, a JAK1 and JAK2 inhibitor, in myelofibrosis. N Engl J Med 363:1117–1127

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Hematology, Department of Internal Medicine, University Hospital Ospedale di Circolo e Fondazione Macchi, Varese, Italy

    Francesco Passamonti

Authors
  1. Francesco Passamonti
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Francesco Passamonti .

Editor information

Editors and Affiliations

  1. Div. Ematologia, Ospedali Riuniti di Bergamo, Largo Barozzi 1, Bergamo, 24128, Italy

    Tiziano Barbui

  2. Dept. Internal Medicine, Div. Hematology, Mayo Clinic, First Street SW. 200, Rochester, 55905, Minnesota, USA

    Ayalew Tefferi

Rights and permissions

Reprints and permissions

Copyright information

© 2012 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Passamonti, F. (2012). Risk Stratification in PMF. In: Barbui, T., Tefferi, A. (eds) Myeloproliferative Neoplasms. Hematologic Malignancies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-24989-1_14

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-24989-1_14

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-24988-4

  • Online ISBN: 978-3-642-24989-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation