What is the Difference Between Primary Polycythemia and Secondary Polycythemia?

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Primary polycythemia and secondary polycythemia are both conditions that result in an increased red blood cell count, but they have different causes and characteristics.

Primary Polycythemia:

  • Caused by an abnormality of the cells in the bone marrow that form red blood cells.
  • Most commonly caused by an acquired genetic mutation in the Janus kinase 2 (JAK2) gene.
  • Results in the overproduction and accumulation of red blood cells due to the mutation expressed within the hematopoietic stem cell or progenitor.

Secondary Polycythemia:

  • Caused by a disorder originating outside of the bone marrow that causes overstimulation of the normal bone marrow, leading to an overproduction of red blood cells.
  • Most commonly associated with low oxygen levels from tissue hypoxia, which can stimulate the kidneys to increase erythropoietin production.
  • The overproduction of red blood cells is due to a number of factors, such as elevated levels of erythropoietin or other transcription factors.

In summary, primary polycythemia is caused by an intrinsic defect in the bone marrow cells, while secondary polycythemia is caused by external factors that lead to the overstimulation of the normal bone marrow. Both conditions result in an increased red blood cell count, but their origins and characteristics differ.

Comparative Table: Primary Polycythemia vs Secondary Polycythemia

Primary and secondary polycythemia are both disorders characterized by an overproduction of red blood cells. However, there are key differences between the two:

Primary Polycythemia Secondary Polycythemia
Also known as polycythemia vera Caused by an underlying condition
Results from a mutation in hematopoietic stem cells Caused by excess production of erythropoietin (EPO) or other factors
Elevated erythropoietin levels Normal or suppressed erythropoietin levels
Increased levels of all three peripheral blood cell lines (RBCs, white blood cells, and platelets) Increased levels of RBCs only
Neoplastic disorder Rare condition
Involves increased proliferation of erythroid progenitor cells Overproduction of RBCs due to an external factor
No intrinsic defect in erythroid progenitor cell lineage Intrinsic defect in erythroid progenitor cell lineage

Some common causes of secondary polycythemia include obstructive sleep apnea, smoking or lung diseases, obesity hypoventilation syndrome, chronic obstructive pulmonary disease (COPD), use of diuretics, and use of performance-enhancement drugs, including EPO, testosterone, and anabolic steroids.