Polycythemia Vera

(P.V.)

By: Jake K.

Period 2

Polycythemia Vera

• Polycythemia Vera or P.V. is a disease where the bone marrow creates too many red blood cells.

• Polycythemia means “many cells in the blood”

• It is a rare or “orphan” disease that occurs once in every 200,000 people

What are some symptoms?

• Some early symptoms include:

• Fatigue • A feeling of fullness or bloating in your left upper

abdomen due to an enlarged spleen

• Chest pain • Dizziness (including headaches)• Blood clots

What are some symptoms?Continued….

• Some major symptoms that include blood clotting are:

• Stroke• Sudden difficulty speaking or understanding speech

(aphasia)

• Sudden numbness, weakness, or paralysis of your

face, arm or leg — usually on one side of your body

How is it Diagnosed?

• For over 100 years P.V. has been known as a disease of exclusions.  That means that the doctors ruled out every other known disease by use of bone marrow biopsies, ultrasounds, CBC’s, and if the patient didn’t have any abnormalities of those, then they deduct it must be P.V.  However, in March 2005, a team of researchers discovered an indicator called JAK 2 (a genetic mutation in the protein tyrosine kinase).  Now doctors just have to order a test to see if the patient carries the JAK2 mutation and if it did, then they know the patient has PV. 

How does P.V affect the body?

P.V. does not affect the external outside of a patients body.

Two kinds:1. Polycythemia- increased amount of red blood

cells in the blood stream

2. Secondary- Polycythemia- limited oxygen due to living at high altitude and/or smoking

Age group

• The age for someone with Polycythemia Vera is from age 20-80. The mean or average age is around 60 years.

60 year old man (Colonel Sanders)

It affects mostly older people, but also young adults and up.

Treatment

• Treatments include:

• Phlebotomy with a baby aspirin (low dose to help thin the blood.)

• Heavier medication such oral chemotherapy - Hydroxyurea

What are Phlebotomies?

Phlebotomies are usually the first option the patient has. It involves a certain amount of blood

(usually averaging one pint) to be drawn from the body. Because the blood is removed from the body, there are less red blood cells to affect the patient. Patients usually have phlebotomies more than once and they can happen on a monthly basis for one’s lifetime.

Any Cures??

Currently, there are seven clinical trials being preformed around the world to test out new medication and to see if any of them will be the key to cure PV.

Because there is no exact cause that doctors have found for P.V, there is no “target” cure or main cure for the disease. Most patients use blood thinners to tone down the red blood cells, but those drugs also tone down the count of others cells such as white blood cells. Those blood thinners are “general” drugs or, not the exact drug that pinpoints red blood cells but all cells.

One Normal Day

• Everyone with PV has it to a certain degree so everyone’s “normal” day is different.

• For my dad, a normal day is taking his morning pills, going for a run, then to work, then at night he takes more pills, and once a month he will take a blood test (CBC) to see if everything is okay.

Is it Expensive?

• The medication for Polycythemia Vera is expensive, but if you’re lucky enough to have insurance, it covers most of the medication.

• With my dad’s splenectomy (removal of the spleen) however, the insurance covered most of the procedure but the overall total amount was still costly for us.

• Overall, insurance covers most of the procedures, medicine, etc. so it’s not typically too costly for our family.

Conclusion

The main reason I chose this disease was because my father has it and in some ways it has changed my family’s life. During my research, I have found that there are two types of Polycythemia Vera. My main source of information was between the MPD Foundation and my dad. I still would like to figure out what causes the disease given the fact it is still unknown. I do believe my downfall would be that there is no exact cause for P.V. and that does take away some great research topics that I could of used. In conclusion I had a blast learning what and how P.V. does and how it affects the people who have it.

Diagram 1

Diagram 1 explanation

• This graph shows the over-production of This graph shows the over-production of red blood cells, white blood cells and red blood cells, white blood cells and blood platelets blood platelets

• Also it shows the B19 Parvovirus Also it shows the B19 Parvovirus Capside that might be the cause of P.V.Capside that might be the cause of P.V.

Diagram 2P.V. Therapies

0.0%

10.0%20.0%30.0%40.0%

50.0%60.0%70.0%

80.0%90.0%

100.0%

Bone MarrowTransplant

Radation Splenectomy Transfusions Phlebotomy Medications

P.V Patients

Mesa et.al. Cancer 2007:109:68 -76

Bibliography

• "canada.com ." canada.com - search. N.p., n.d. Web. 14 Mar 2010. <http://polycythemiaveraproducts.com/ html>.

• "Medline Plus." Polycythemia vera: Medline Plus Medical Encyclopedia. N.p., n.d. Web. 14 Mar 2010. http://www.nlm.nih.gov/medlineplus/ency/article/000589.htm.

• "Polycythemia Vera." Polycythemia Vera. N.p., n.d. Web. 14 Mar 2010. http://www.som.tulane.edu/classware/pathology/Krause/PV/PV.html.

• "Polycythemia vera." Polycythemia vera - MayoClinic.com. N.p., n.d. Web. 14 Mar 2010. <http://www.mayoclinic.com/health/polycythemia-vera/DS00919>.

• K., Sam. Intervew by Jake Klepper. 14 Mar 2010. Print.

Interview