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

Upcoming Events


Camp U.O.T.S. is an annual weeklong, overnight camp for children with cancer and blood disorders who are treated at Nicklaus Children’s Hospital.

Learn more and register

From the Newsdesk

Neuro Oncology Holiday Party 2017
12/15/2017 — Children being treated by the Cancer & Blood Disorders Center, Neuro Oncology Program at Nicklaus Children’s Hospital and their families took part in a holiday celebration.
Talkin' Kids Health - Discussing Brain Tumors
11/29/2017 — Dr. Toba N. Niazi, Neurosurgeon, and Dr. Ziad A. Khatib, Hematologist and Oncologist, discuss the second leading cause of cancer in children, brain tumors.



Meet our July Patient of the Month, Lacy. Lacy was only 2 years old when her parents noticed that something was wrong. They took her to various doctors to try to find what could be the cause, Lacy had an 8 cm. tumor in her brain, occupying most of the lower part of her head.