Also known as: Polycythemia vera, primary polycythemia, secondary polycythemia, newborn polycythemia, PFCP.

What is polycythemia?

Polycythemia is a rare and serious blood disease that causes the bone marrow to produce too many red blood cells (adjusted for race, age, sex and altitude) to circulate in the blood stream. This causes the blood to become thicker, makes it more difficult for blood to flow throughout the body, and can lead to blood clots. 

Polycythemia can be divided into:

  • Primary or intrinsic polycythemia
  • Secondary or extrinsic polycythemia

Primary polycythemia has three forms:

  1. Newborn polycythemia
  2. Primary familial/congenital polycythemia (PFCP)
  3. Polycythemia vera
Secondary polycythemia may be congenital (e.g.hemoglobin variants) or acquired.

What causes polycythemia? 

Primary polycythemia is caused by an acquired or inherited gene mutation (change). Secondary polycythemia is due to outside factors like lack of oxygen from lung cardiac, kidney, or liver disease, high altitude or abnormal hemoglobins (in the newborn baby). These all stimulate erythropoiesis (red cell production).

What are the symptoms of polycythemia? 

Children with polycythemia frequently have no symptoms. When present, common symptoms include; Red/purple color, feeding problems, headaches, trouble breathing, dizziness, itchiness, and weakness/tingling of hands and feet.

What are polycythemia care options? 

In children with polycythemia, the thickness of the blood can be reduced with a treatment called phlebotomy (withdrawing blood from a vein). Low dose aspirin and other medications can reduce the number of red cells. 

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 5/24/2018 9:35:25 AM

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