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Encyclopedia of Cancer pp 1–4Cite as

Primary Myelofibrosis

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Synonyms

Agnogenic myeloid metaplasia; Chronic idiopathic myelofibrosis; Idiopathic myelofibrosis; Myelofibrosis with myeloid metaplasia

Definition

Primary myelofibrosis (PMF) is a stem cell-derived clonal myeloproliferative disorder (MPD) that is characterized clinically by anemia, marked enlargement of the spleen and liver, and severe constitutional symptoms. Peripheral blood findings include the presence of immature myeloid cells including nucleated red blood cells, immature granulocytes, and teardrop-shaped erythrocytes. The bone marrow histology exhibits reticulin and collagen fibrosis, osteosclerosis, and angiogenesis.

Characteristics

Background

The blood and bone marrow features associated with PMF are discovered either de novo (i.e., PMF) or in the setting of either polycythemia vera (post-PV MF) or essential thrombocythemia (post-ET MF). PMF is also known by many synonyms. However, the use of the term “PMF” was recently endorsed by the International Working Group for...

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References

  • Arana-Yi C, Quintas-Cardama A, Giles F et al (2006) Advances in the therapy of chronic idiopathic myelofibrosis. Oncologist 11:929–943

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  • Tefferi A (2005) Pathogenesis of myelofibrosis with myeloid metaplasia. J Clin Oncol 23:8520–8530

    Article  CAS  PubMed  Google Scholar 

  • Tefferi A (2006) New insights into the pathogenesis and drug treatment of myelofibrosis. Curr Opin Hematol 13:87–92

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  • Tefferi A, Jimenez T, Gray LA et al (2001) Radiation therapy for symptomatic hepatomegaly in myelofibrosis with myeloid metaplasia. Eur J Haematol 66:37–42

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  • Visani G, Finelli C, Castelli U et al (1990) Myelofibrosis with myeloid metaplasia: clinical and haematological parameters predicting survival in a series of 133 patients. Br J Haematol 75:4–9

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Author information

Authors and Affiliations

  1. Division of Hematology, Mayo Clinic College of Medicine, Rochester, MN, USA

    Ayalew Tefferi

Authors
  1. Ayalew Tefferi
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Corresponding author

Correspondence to Ayalew Tefferi .

Editor information

Editors and Affiliations

  1. Abt. Tumorgenetik, Deutsches Krebsforschungszentrum, Heidelberg, Germany

    Manfred Schwab

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© 2015 Springer-Verlag Berlin Heidelberg

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Tefferi, A. (2015). Primary Myelofibrosis. In: Schwab, M. (eds) Encyclopedia of Cancer. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-27841-9_4740-2

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  • DOI: https://doi.org/10.1007/978-3-642-27841-9_4740-2

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Online ISBN: 978-3-642-27841-9

  • eBook Packages: Springer Reference Biomedicine and Life SciencesReference Module Biomedical and Life Sciences

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