Publisher: Kenneth Kee
Publication Date: May 11, 2016
Binding: Kobo eBook
What is Polycythemia Vera?
Polycythemia vera (PV) is a medical bone marrow disorder in which there is an uncontrolled proliferation of the blood cells.
All the blood cells in the body are involved.
Polycythemia rubra vera (PRV) mainly produces too many red blood cells.
The number of white blood cells and platelets may be higher as well.
It is believed to be a benign form of neoplasm giving rise to a chronic disease from which the patient eventually dies.
What is the cause of Polycythemia vera?
The exact cause is not known but it is often linked to a gene defect called JAK2V617F.
The reason behind this gene defect is not known.
Polycythemia vera (PV) is produced by somatic mutation in a single hematopoietic stem cell.
This mutation can be investigated only in female patients, as it makes use of polymorphisms on the X-chromosome and takes advantage of its inactivation.
The red cells are normally responsive to erythropoietin but they do not depend upon it to multiply.
There is a danger of transformation to acute myeloid leukemia.
PV is a myeloproliferative disorder with mainly erythrocyte hyperplasia, but also myeloid leukocytosis, thrombocytosis and splenomegaly
Polycythemia Primary Causes
In primary polycythemia, inherited or acquired problems with red blood cell production result in polycythemia.
There are two main disorders that place it in the primary polycythemia category:
1. Polycythemia vera (PV) or polycythemia rubra vera (PRV)
Polycythemia vera (PV) is linked to a genetic mutation in the JAK2 gene which is believed to raise the sensitivity of bone marrow cells to a hormone called erythropoietin (Epo), leading to higher red blood cell production.
The levels of other forms of blood cells (white blood cells and platelets) are also often higher in this disorder.