Polycythemia and oxygen sensing

Pathologie-biologie
Jey Maran, Josef T Prchal

Abstract

Polycythemias can be differentiated based on the responsiveness of erythroid progenitors to circulating cytokines. Primary polycythemias are characterized by an augmented response due to acquired somatic or inherited germ-line mutations that are expressed within hematopoietic progenitors causing increased proliferation or decreased apoptosis and resulting in accumulation of red blood cells. In terms of oxygen requirements, primary polycythemias can be viewed as the production of hemoglobin fully dissociated from the tissue oxygen needs and from the oxygen sensing pathway. Polycythemia vera (PV) is the most common primary polycythemia. PV bone marrow progenitors cells can form erythroid colonies in the absence of exogenous erythropoietin in vitro. These endogenous erythroid colonies (EEC) are useful in differentiating PV and secondary polycythemias. They also can differentiate PV where this feature is independent of Epo signalling from primary familial and congenital polycythemia. In this autosomal dominant primary polycythemia, at variance with PV, EEC formation is abolished by anti-Epo and anti-Epo receptor neutralising antibodies. Mutations of the EPOR have been described and resulted in nine cases in truncated EPORs lacking ...Continue Reading

References

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Citations

May 1, 1976·Annals of Internal Medicine·M A LichtmanJ W Adamson
Jun 13, 1974·The New England Journal of Medicine·Jaroslav F Prchal, A A Axelrad
Dec 31, 1997·The Journal of Clinical Investigation·M MrugJaroslav F Prchal
Feb 7, 2001·Proceedings of the National Academy of Sciences of the United States of America·V DivokyJaroslav F Prchal
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May 4, 2002·Blood Cells, Molecules & Diseases·Sonny O AngJosef T Prchal
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Nov 26, 2003·Blood Cells, Molecules & Diseases·Katerina JedlickovaJosef T Prchal

Related Concepts

Polycythemia Vera
Neutralising Antibodies Analysis
Biochemical Pathway
Dioxygen
Germ-Line Mutation
Colony-Forming Units, Hematopoietic
Epoetin Alfa
Somatic Mutation
Erythropoietin Measurement
Bone Marrow

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