PV and fatigue

I was diagnosed 3 years ago with secondary (non cancerous) PV. I do not have the JAK2 mutation. My blood counts have been all over the map which concerns me. I have had phlebotomy on a three month schedule for the time period. I am always exhausted, short of breath and weak. Really frustrating from someone who used to walk 6 miles a day without complaint.
I am concerned with how this is going to progress. My mom had Myelofibrosis and her mom died of acute myelogenous leukemia. Mom's doctor suggested that it would possibly run in families, but my doctor disagrees.
My question is can I still develop JAK2 mutation? Trying to figure out where to go from here.

I was diagnosed 3 years ago with secondary (non cancerous) PV. I do not have the JAK2 mutation. My blood counts have been all over the map which concerns me. I have had phlebotomy on a three month schedule for the time period. I am always exhausted, short of breath and weak. Really frustrating from someone who used to walk 6 miles a day without complaint.
I am concerned with how this is going to progress. My mom had Myelofibrosis and her mom died of acute myelogenous leukemia. Mom's doctor suggested that it would possibly run in families, but my doctor disagrees.
My question is can I still develop JAK2 mutation? Trying to figure out where to go from here.

Hi @casevin7 JAK2 is an acquired mutation so it is possible to develop it in the future. But it’s not something to dwell on. It would drive us all crazy trying to predict what mutation could happen in any of our millions of cells.

While the JAK2 mutation isn’t passed down generationally it possibly may run in families. The suggestion from some research is that there “could” be a link through generations of being potentially genitally susceptible or there could be a vulnerability in a genetic pattern that might run in families. Mostly they’re just random: a stray gamma ray passing through the earth, a DNA strand that didn’t split perfectly, who knows… but my hematolgist said it perfectly “S*** happens” and there’s just no point dwelling on the hows and whys.
https://www.healthline.com/health/myelofibrosis/what-is-primary-myelofibrosis#symptoms
With your familial history of blood cancers it’s pretty reasonable to want to know what could potentially happen down the road. Would you be open to getting a second opinion?

Are you following any treatment plan besides the occasionally phlebotomy? Any aspirin therapy or Hydroxyurea?

Has anyone found help for the itching?

Thanks for the reply. I am trying to find answers. I am on my second oncologist/hematologist. I really liked the first one but he moved two states away. I also live in a small town with limited access to doctors. I tried to get into MD Anderson in Houston but was denied access.
I tried the aspirin route but it was too hard on my stomach so I had to quit. I am not on any other meds for this condition as my doctor seems to believe that I do not have it. But my symptoms are all listed on PV sites. I did not know I could get a video appointment so might look into that.
I am due for labs tomorrow and expect a phlebotomy next week since my hematocrit is always high. And my symptoms are pretty unbearable just now. They usually improve slightly after the blood draw.

I don’t have sleep apnea, my kidney functions are good and my spleen is good.

Hi @jerrlin. In your case, you have the JAK2 mutation which caused the PV so it’s not considered secondary to another cause such as those I listed regarding EPO. @casevin7 doesn’t have the mutation but was diagnosed with secondary PV which is generally from an underlying factor.

I was diagnosed 3 years ago with PV and positive for Jak 2. I take 2 baby aspirin a day and a phlebotomy about every 3 months. The fatigue has been coming more often and the itching is a real problem at times. Just wondering if anyone also has shortness of breath? I have no other health problems and take no other medication. I’m very active but find this sometimes getting in my way.

Good morning, @casevin7 It’s awful to feel so miserable. This sounds so disrupting to your normal, active life style. When you were initially diagnosed with secondary PV did you have a bone marrow biopsy? How were you diagnosed?

Do you recall if you’ve had a blood test for EPO? Erythropoietin is a hormone that tells the stem cells in the bone marrow to make more red blood cells. EPO is made by cells in the kidney and these cells release more EPO when blood oxygen is low.

One of the causes can be sleep apnea. Lung, heart and kidney disease can also be underlying culprits.
This article on erythrocytosis from the Cleveland Clinic might be useful information for you.
https://my.clevelandclinic.org/health/diseases/23468-erythrocytosis
When’s the last time you’ve been seen by your doctor? Have they checked for a swollen spleen by pushing in your upper left abdominal area? This is always one of the first things my oncologist does.
Are your other blood result numbers off such as your kidney function? Do you know if you have sleep apnea?