Investigation and Management of Erythrocytosis

Curr Hematol Malig Rep. 2016 Oct;11(5):342-7. doi: 10.1007/s11899-016-0334-1.


An absolute erythrocytosis is present when the red cell mass is greater than 125 % of the predicted value for sex and body mass. It can have a primary or secondary and congenital or acquired cause. New causes particularly congenital continue to be discovered and investigated. Investigation for the cause starts with repeat and confirmation of the raised hemoglobin and measurement of an erythropoietin level to indicate whether to pursue primary or secondary causes and then further investigations as appropriate. Management options include low dose aspirin and venesection. Specific management advice is available for certain specific clinical situations.

Keywords: Erythrocytosis; Management; Myeloproliferative disorders; Treatment.

Publication types

  • Review

MeSH terms

  • Adaptor Proteins, Signal Transducing
  • Aspirin / therapeutic use
  • Erythropoietin / metabolism
  • Humans
  • Intracellular Signaling Peptides and Proteins
  • Mutation
  • Phlebotomy
  • Polycythemia / diagnosis*
  • Polycythemia / genetics
  • Polycythemia / therapy
  • Polycythemia Vera / genetics
  • Polycythemia Vera / pathology
  • Proteins / genetics
  • Receptors, Erythropoietin / genetics
  • Von Hippel-Lindau Tumor Suppressor Protein / genetics


  • Adaptor Proteins, Signal Transducing
  • Intracellular Signaling Peptides and Proteins
  • Proteins
  • Receptors, Erythropoietin
  • SH2B3 protein, human
  • Erythropoietin
  • Von Hippel-Lindau Tumor Suppressor Protein
  • VHL protein, human
  • Aspirin