Polycythemia Vera

Jun 29, 2023
White Blood Cells origin

Polycythemia Vera (PA)/ Erythrocytosis:

What is Polycythemia Vera?
Why uncontrolled red blood cells are produced in polycythemia Vera?

Polycythemia Vera is a blood disorder, where in the red blood cells formation is abnormally higher. Polycythemia Vera is a rare blood cancer, It is caused due to mutation in the gene Janus kinase-2 (JAK2), due to which a neoplastic growth of hematopoietic progenitor stem cells occurs. Red blood cells and other types of blood cells are produced by hematopoietic progenitor stem cells in bone marrow. Neoplastic growth of hematopoietic progenitor stem cells keeps on producing higher red blood cells. Even other blood cells are also produced in excess along with RBCs while RBC are predominantly produced in higher numbers in PA.

What happens after higher red blood cells count?

Blood thickness increased due to increase in blood corpuscles. Increased blood cells cause increase in blood volume and increase in pressure and increased resistance to blood flow. Emboli formation, blood clot formation, blocking arteries and veins. It can lead to cardiovascular disturbances, even congestive cardiac arrest and stroke.

Symptoms of Polycythemia Vera.

Fatigue and weakness, lack of energy, numbness in body parts, gout.
Difficulty for taking normal breath.
Excessive Menstrual Bleeding.
Increased Blood Pressure
Blurred or Double Vision
Inability to concentrate
Sweating at night.
Flushing face.
Bleeding from gum, and nose.
Blood through coughing.

Treatment of Polycythemia Vera.

1) Phlebotomy :

In PA increased blood cells is the root cause for any complications therefore one of the easy and faster ways to treat it is removing some Blood from the patient. The procedure is called phlebotomy.
2) Medicines / Chemotherapy: Medicines which lower the formation of blood cells which directly act in bone marrow stem cells. Example Hydroxyurea.

3) Interferon treatment: alfa-2b
4) Janus kinase inhibitor (JAK inhibitor) : Ruxolitinib

5) Alkylating agent: Busulfan.

Secondary polycythemia:

It’s caused by other reasons which are not associated with mutations in any gene.

Person with Cyanotic heart diseases with right-to-left shunts.

People who live in high altitudes develop asymptomatic polycythemia.

Lungs disease like COPD.

Sleep apnea. Pickwickian syndrome.

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