Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Primary Myelofibrosis

Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_4740-2

Synonyms

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...

Keywords

Chronic Myeloid Leukemia Graft Versus Host Disease Essential Thrombocythemia Allogeneic Stem Cell Transplantation Extramedullary Hematopoiesis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

  1. Arana-Yi C, Quintas-Cardama A, Giles F et al (2006) Advances in the therapy of chronic idiopathic myelofibrosis. Oncologist 11:929–943CrossRefPubMedGoogle Scholar
  2. Tefferi A (2005) Pathogenesis of myelofibrosis with myeloid metaplasia. J Clin Oncol 23:8520–8530CrossRefPubMedGoogle Scholar
  3. Tefferi A (2006) New insights into the pathogenesis and drug treatment of myelofibrosis. Curr Opin Hematol 13:87–92CrossRefPubMedGoogle Scholar
  4. Tefferi A, Jimenez T, Gray LA et al (2001) Radiation therapy for symptomatic hepatomegaly in myelofibrosis with myeloid metaplasia. Eur J Haematol 66:37–42CrossRefPubMedGoogle Scholar
  5. 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 75:4–9CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Division of HematologyMayo Clinic College of MedicineRochesterUSA