PMID: 9549927Apr 29, 1998Paper

Pathogenesis of polycythemia vera

Haematologica
J L Fernández-LunaA Benito

Abstract

Polycythemia vera (PV) is an acquired clonal myeloproliferative disorder characterized by increased production of mature red cells. We still lack a molecular target responsible for this disorder; however, recent investigations have focused on a number of molecules involved in signal transduction pathways mediated by erythropoietin (Epo) and other growth factors. Here we review the implication of these molecules in the pathogenesis of PV. The material reviewed in this work includes articles published in journals covered by Medline. We also include data obtained in our laboratory regarding to the significance of apoptosis inhibitory proteins in erythroid development. Overproduction of erythroid cells in PV is particular in that it occurs in the absence of a recognizable physiologic stimulus, since circulating serum levels of Epo are normal or lower than normal. Genetic analysis as well as in vitro studies, have established an essential role for Epo in the survival and maturation of committed erythroid progenitors. Epo initiates its cellular response by binding to the Epo receptor (EpoR) expressed on the surface of immature erythroblasts. Following ligand binding, EpoR is known to activate a cytoplasmic protein tyrosine kinase, Ja...Continue Reading

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