Polycythemia Vera in Children
What is polycythemia vera?
Polycythemia vera is a serious, but very rare blood disorder in children. With polycythemia
vera, the bone marrow makes too many red blood cells. The extra cells make the blood
too thick. This may lead to blood clots. The clots can decrease the blood supply to
organs, tissues, and cells.
What causes polycythemia vera?
An abnormal gene causes polycythemia vera. Although it is more common in some families,
it is not passed from parents to children.
What are the symptoms of polycythemia vera?
Some children do not have symptoms and polycythemia vera may be found when they're
having blood tests for other reasons. Symptoms occur more often in adults. The following
are common symptoms of polycythemia vera. However, each child may experience symptoms
differently. Signs and symptoms may include:
- Feeling weak and tired
- Trouble breathing
- Enlarged spleen
- Trouble seeing
- Itching or burning
- Reddish coloring of face
- Bleeding gums
- Losing weight
The symptoms of polycythemia may look like other conditions or medical problems. Always
consult your child’s doctor for a diagnosis.
How is polycythemia vera diagnosed?
The following blood tests are used in the diagnosis of polycythemia vera.
- Hemoglobin and hematocrit. It measures the amount of hemoglobin and red blood cells in the blood.
- Complete blood count, or CBC. A complete blood count checks the red blood cells, white blood cells, blood clotting
cells (platelets), and sometimes, young red blood cells (reticulocytes). It includes
hemoglobin and hematocrit and more details about the red blood cells.
- Peripheral smear. A small sample of blood is examined under a microscope. Blood cells are checked to
see if they look normal or not.
- Erythropoietin (EPO) level. This test measures the amount of EPO in the blood. EPO is a hormone involved in red
blood cell production.
How is polycythemia vera treated?
Your child’s healthcare provider will figure out the best treatment based on:
- How old your child is
- His or her overall health and medical history
- How sick he or she is
- How well your child can handle specific medicines, procedures, or therapies
- How long the condition is expected to last
- Your opinion or preference
Your child's health care provider will likely refer you to a hematologist, a specialist
in blood disorders. Treatment may include:
- Venipuncture or removing some blood, thus reducing the number of red blood cells (similar
to a person donating blood)
- Low-dose of aspirin to prevent blood clots
- Medications to decrease the production of red blood cells in the bone marrow
- Other treatments are being studied
What are the complications of polycythemia vera?
Complications depend on how severe the polycythemia is. They include:
- Blood clots in the veins of the liver
- Deep vein thromboses or blood clots in the veins
- Heavy bleeding
How is polycythemia vera managed?
Your child will be monitored for changes in blood test results or signs and symptoms
of complications. Your child should avoid extreme heat, like hot baths. Heat may make
some symptoms like itching and burning worse. And he or she should stay away from
sports and activities that are likely to cause injuries. Injuries may increase the
risk of bleeding.
When should I call my child's healthcare provider?
Call your child's healthcare provider if you notice new or worsening symptoms. For
example, call if you see that your child is more tired than usual. Call your child's
healthcare provider or seek medical care if your child:
- Is injured
- Has pain in his or her arms or legs or abdomen (may be from a blood clot)
Key points about polycythemia vera
- Polycythemia vera is blood disorder in which the bone marrow produces too many red
blood cells. The extra cells make the blood too thick, increasing the risk of blood
- Blood clots may cause serious problems depending on where they form. The clots can
decrease the blood supply to the heart, brain, or liver, for example.
- Treatment may include venipuncture to remove blood and taking aspirin.
Tips to help you get the most from a visit to your child’s healthcare provider:
- Before your visit, write down questions you want answered.
- At the visit, write down the names of new medicines, treatments, or tests, and any
new instructions your provider gives you for your child.
- If your child has a follow-up appointment, write down the date, time, and purpose
for that visit.
- Know how you can contact your child’s provider after office hours. This is important
if your child becomes ill and you have questions or need advice.