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...
KeywordsPolycythemia Vera Erythroid Progenitor JAK2V617F Mutation Secondary Erythrocytosis Secondary Polycythemia
- Tefferi A (2006) Classification, diagnosis and management of myeloproliferative disorders in the JAK2V617F era. Hematology Am Soc Hematol Educ Program 2006:240–245Google Scholar