Polycythemia Vera

Disclaimer:

I have found some of this information to be personally helpful to me. I have several genetic predispositions that put me at high risk to acquire the JAK2 mutation.

Additionally, some of this information I have not yet had a chance to test or validate on myself yet. Finally, some of this information is ONLY conjecture based on yet unproven hypothesis based on connected the dots of data between various medical disciplines. ALL INFORMATION IS PROVIDED FOR EDUCATIONAL INFORMATION ONLY.

Additional IMPORTANT legal information can be found at the bottom of this page.

 

Polycythemia Vera

Is defined as a slow growing blood cancer that is considered a Myeloproliferative Neoplasm.  To put it plainly, it is a genetic blood cancer that is in the class of blood disorders known as MPNs, which is when your body creates to many of a certain blood cell. However in POLYcythemia Vera, the body creates too many of ALL blood cells.

 

Diagnosis

Diagnosis is made primarily by genetic testing.

Diagnosis is made by testing look to see if there are any genetic mutations in the JAK2 signaling pathway, specifically JAK2 V617F mutation.

JAK2 V617F is located at 

rs77375493
GG = Good
TT = Bad

This SNP encodes for the JAK2 V617F mutation.

V617F means in the JAK2 signaling gene the amino acid located at position 617 is changed from a Valine to a Phenylalanine molecule. [R1, R2, R3, R4, R5, R6]

 

Genetic Risks Factors

G allele of JAK2 rs10974944 strongly associated with V617F [R1,R2]
p = < 0.0001 for both GG and CG
p = < 0.000001 for GG

JAK2 GGCC 46/1 Haplotype is a risk factor for developing a JAK2 V617F mutation [R1]

  • rs3780367
  • rs10974944
  • rs12343867
  • rs1159782
  • both V617F and 46/1 rs12340895
  • Crohns rs10758669

Other Risk Factors

 

Germ line variants predispose to both JAK2 V617F clonal hematopoiesis
rs59384377
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5085254/

Germline Variations in JAk2, TERT, HBS1L-MYB and MECOM
JAK2 46/1 rs12343867
JAK2 intron 8 rs12339666
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5652698/

JAK2 V617F (1849 G > T) In Cervical Cancer Related to HPV and STIs
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6037210/

Genetic variation at MECOM, TERT, JAK2
rs12339666 (if V617F negative)
rs9376092 (if V617F positive more like to have ET with the rs9376092 mutation)
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4396373/

Genetic association between JAK2 polymorphisms in Hong Kong Chinese
rs12342421
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4293821/

C allele of JAK2 rs4495487 is an additional candidate locus that contributes to MPN predisposition in the Japaniese
https://www.ncbi.nlm.nih.gov/pubmed/22251709


nullizygosity for the JAK2 46/1 haplotype is associated with inferior survival.
https://www.ncbi.nlm.nih.gov/pubmed/19847199

 

Taurine

Taurine is likely helpful for 2 reasons.

  1. To improve blood flow (likely secondary to its cytoprotective effects)
  2. As a Cytoprotective agent
    1. According to Wikipedia:
      “Cytoprotection is a process by which chemical compounds provide protection to cells against harmful agents.”
  3. As an anti-inflammatory (again likely secondary to it’s cytoprotective effects)
    1. Taurine has recently been shown to help in:
      1. Arthitis
      2. Chronic Prostatis
      3. Improving sleep
      4. Increase the rate of which an infection is cleared.

References:
Ripps, Harris, and Wen Shen. “Review: taurine: a “very essential” amino acid.” Molecular vision vol. 18 (2012): 2673-86.


Schaffer, Stephen, and Ha Won Kim. “Effects and Mechanisms of Taurine as a Therapeutic Agent.” Biomolecules & therapeutics vol. 26,3 (2018): 225-241. doi:10.4062/biomolther.2017.251

 

 


 

About PV and Cause of PV Symptoms

You are Infected 

(The story behind Demodex, Acne, Cysts, Bumps, Dots and Other Skin Abnormalities)

So you need to know that here is a small bug on your face right now. The bug is called:

Demodex

Demodex is considered a “normal and harmless” ectoparasite and according to most medical journals. However recent evidence has been showing more and more that this nasty guy is responsibile for:

  • Roscacia
  • Acne
  • Cystic Acne
  • Facial Cysts
  • Acne Comedones
  • Whiteheads
  • Blackheads
  • and more

 

Demodex comes in many forms however there are only 2 that infect humans

Demodex folliculus 

Demod

 

Blood and Blood Vessels

So many of the PV symptoms we experience is due to the overabundance of NOT ONLY BLOOD but BLOOD VESSELS. The amount of blood we have in our bodies affects angiogenesis (or the growth of new blood vessels). Angiogensis is at least in part regulated by blood pressure, blood volume, and certain blood cells. Since individuals with PV have a greater blood volume, there is a chance they have a greater number of blood vessels throughout their body since the conditions were present for it to happen.

 

Angiotensin 2

“Angiotensin II is produced locally within the kidney and mediates tissue injury through a series of nonhemodynamic effects. angiotensin II is not only involved in the regulation of blood pressure, water and sodium homeostasis, and control of other neurohumoral systems, but also leads to excessive production of reactive oxygen species and to hypertrophy, proliferation, migration, and apoptosis of vascular cells.” [R]

 

Blood Cells, Angiogenesis

So lets look at your feet and your stomach.

Feet

Your feet are the furthest from your heart and therefor it takes the most amount of work to get blood back to your heart. Additionally more blood may be delivered to your feed than other parts of your body. Therefore there will likely be more vascular epitheial growth factor and more angiotensin which may be implicated in some of the symptoms of PV as more blood vessels could be forming thus resulting in increased blood volume and increased RBC volume in that region thus resulting in an increased cytokine response.

Stomach

Due to the increased angiogenisis and increased amount of RBC individuals with PV may have an increased inflammatory response to pathogens.

This is because cytokines are released from RBC, therefore if there are more RBC there will likely be an increased cytokine response.

Details

Blood vessel growth is in part due to the role of angiotensin II and Vascular endothelial growth factor (VEGF). VEGF drives the process and angiotensin II helps move it along. Your body has an almost constant supply of Angiotensin II through the RAAS system. RAAS or Renin Angiotensin Aldosterone system. Angiotensinigen is released from the liver and sits inactive in the blood. But AS soon as your body detects a significant change in blood pressure your kidneys express Regnin. Renin basically acts like Pac Man and cuts Angiotensinegen down from a 10 chain amino acid down to a 9 chain amino acid known as Angiotensin I. 


Then almost instantly angiotensin I interact with Angiotensin-Converting Enzyme (ACE) that lines your lungs and blood vessel walls.  ACE removes yet another amino acid and creates Angiotensin II. Angiotensin II is instantly absorbed into the blood vessel causing it to constrict. [R]

Angiotensin I and II are both implicated in the role of blood vessel creation, but Angiotensin I appears to have a slight inhibitory effect, whereas Angiotensin II active aids angiogenesis.  [R]

Angiotensin II is the same molecule your body uses to quickly regulate blood pressure drops. (See note further down on why IV therapy and its interaction with the RAAS system can affect PV symptoms for the better.) [R]

Treatments options for excessive ACE activity or for ANG2 related symptoms could possible include:

  • ACE Inhibitors (if blood pressure is not low)
  • IV Hydration Therapy (ideally a Lactated Ringer solution)

IV Therapy Information

IV Hydration Therapy in general and IMMEDIATELY after phlebotomy can help you with SEVERAL symptoms associated with polycythemia vera.

This prevents your blood pressure from dropping as much and consequently tyour body then will NOT release Renin so you will NOT convert angiotensin into angiotensin I.

This should in theory help prevent too many new blood vessels from being created and therefore may reduce the progression of some symptoms.

 

Some Links for PV information

This will be moved in the future this is just here for reference as I develop this page.

https://www.ncbi.nlm.nih.gov/pubmed/22374305

https://www.nhlbi.nih.gov/health-topics/polycythemia-vera

https://www.ncbi.nlm.nih.gov/medgen/45996

 

Cause of PV Symptoms

https://www.ncbi.nlm.nih.gov/pubmed/14631544

 

Taurine and PV Symptoms

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3501277/

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5933890/

 

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2994373/

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3253515/

 

IMPORTANT Legal Disclaimer

NOTHING ON THIS PAGE SHOULD BE CONSIDERED MEDICAL ADVICE. I AM NOT A DOCTOR. 

I do not have any formal healthcare training or education. Please consult with your doctor or healthcare practitioner before you change anything regarding your health.

ALL INFORMATION IS PROVIDED FOR EDUCATIONAL INFORMATION ONLY. While I attempt to keep my recommendations as harmless as possible, IF YOU ATTEMPT ANYTHING LISTED ON THIS PAGE YOU DO IT AT YOUR OWN RISK!

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