Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab


  • Fulvio Della Ragione
  • Silverio Perrotta
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_4668-2



A condition in which the hemoglobin amount and the erythrocytes count are increased over the normal values.


The Greek term polycythemia (literally, many cells in the blood) corresponds, in the clinical setting, to the word erythrocytosis. This condition exists when hemoglobin amount and the erythrocytes count are increased as the consequence of a buildup of the total volume of red cells.

It is possible to distinguish primary and secondary forms as well as congenital or acquired polycythemias.

Primary polycythemias show an enhanced capability of the erythroid progenitors to respond to normal levels of circulating cytokines as the consequence of genetic mutations (both germline inherited and somatically acquired) that cause an increased proliferation or a decreased apoptosis of erythrocytes precursors. From a functional point of view, in these forms, there is a dissociation between the total oxygen-carrying ability (i.e., the total...


Polycythemia Vera Erythroid Progenitor JAK2V617F Mutation Secondary Erythrocytosis Secondary Polycythemia 
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.
This is a preview of subscription content, log in to check access.


  1. Ang SO, Chen H, Hirota K et al (2002) Disruption of oxygen homeostasis underlies congenital Chuvash polycythemia. Nat Genet 32:614–621CrossRefPubMedGoogle Scholar
  2. Gordeuk VR, Stockton DW, Prchal JT (2005) Congenital polycythemias/erythrocytoses. Haematologica 90:109–116PubMedGoogle Scholar
  3. James C, Ugo V, Le Couedic JP et al (2005) A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia vera. Nature 434:1144–1148CrossRefPubMedGoogle Scholar
  4. Khan J, Wei JS, Ringnér M et al (2001) Classification and diagnostic prediction of cancers using gene expression profiling and artificial neural networks. Nat Med 7:673–679CrossRefPubMedPubMedCentralGoogle Scholar
  5. Tefferi A (2006) Classification, diagnosis and management of myeloproliferative disorders in the JAK2V617F era. Hematology Am Soc Hematol Educ Program 2006:240–245Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Department of Biochemistry and BiophysicsSecond University of NaplesNaplesItaly
  2. 2.Department of PediatricsSecond University of NaplesNaplesItaly