ET? PV? Neither?

[Healthy6386]

Hello everyone, I’m confused by my blood tests & blood results & not sure what to do. I went to get my blood drawn for a physical & it showed high RBC, High HGB, and high HCT. I do live in high altitude in Utah. My white blood cells were 10 & platelets were 400 so my GP sent me to a hematologist who said hmmm maybe PV, but we will check. (He seemed to doubt an MPN) So in the meantime I had one phlebotomy that dramatically lowered my HCT , RBC’S & HGB into a normal range. My platelets went from 400 to 360 then 4 weeks check everything was still in range, but my platelets went down to 315 on their own. Would that pretty much eliminate ET? I only ask cause my heam sent me for a JAK2 test which was negative & EPO was 8.8. I’ve never had platelets that weren’t outside of the normal range so idk

[lynnieb]

My haematologist told me to ignore Jak2 as it is only to show whether your ET is idiopathic or not. Not sure about your results as we have different way of doing them in the UK. My platelets are 1400 and I'm Jak2-ve. It is a minefield though, wherever you live, trying to get an answer!

[Mirror368 in reply to lynnieb]

I looked at your profile. It seems your platelets have been very high for years. Do you take low dose aspirin to help avoid clots? Are you taking anything to lower your 1400 platelet count?

Best wishes, Eileen

[Meatloaf9]

Hi, I think the answer to your question can only come from a hematologist who is well versed in MPN's or preferably a MPN specialist. There must have been some reason that your doc ordered a phlebotomy for you. My platelets fluctuated for 10 years before I was diagnosed with ET Jak2 + and 3 years later it transformed to PV. Most PV patients are Jak2+. Good luck to you in getting an answer. Best.

[Silveryeam2]

I found out mine were above 1000 7 yrs ago. I started hydrea and they went down to 800. The dose was increased and they went to 675, but I started to feel some side effects. My white cells stayed extremely low and I became anemic. So 6yrs into this I on my own decided to stop treatment all together. My platelets remain between 1000-1300. But I am not anemic and my white blood cells are normal. I feel better. I decided to pray each day that my Lord would take care of this issue for me. I feel my life is not shorten by this condition ET. Meds make me feel awful so I rather feel good and let my body decide what it wants to do. Sometimes we just get tired of the treatments. Hope everything works out for you as it does for many.

[Mirror368 in reply to Silveryeam2]

I hope you are taking a low dose aspirin to help prevent clots. I am taking 500 mg HU on Monday/Wednesday/Friday, I feel much better this way, My next CBC is in July.

Eileen

[monarch5000]

I'm not a doctor, but I would say neither. Your EPO of 8.8 pretty much rules out PV, and might actually help confirm secondary polycythemia (not a blood cancer) caused (maybe) by the high altitude. Try getting help from this group: facebook.com/groups/5701314...

[hunter5582]

What you are describing sounds like polycythemia, not thrombocytosis. The question would be whether the polycythemia is primary or secondary. While there is a small number of people with PV who are JAK2 negative, this is quite rare. It is worth noting that the different JAK2 tests have different levels of sensitivity. It might be worth checking on that, but is not likely the issue.

There is a diagnostic workup indicted in this situation. Here is one description of that process. emedicine.medscape.com/arti... Suggest contacting a MPN Specialist for a second opinion as well. mpnforum.com/list-hem./

For what it is worth, it sounds like you may be experiencing a secondary polycythemia; however, I would not assume that is what it is. A complete assessment by a MPN-expert hematologist would be indicted in this situation.

Wishing you clear answers ASAP.

[Healthy6386 in reply to hunter5582]

Thanks Hunter. My blood was sent over to a hospital with a PCR JAK2 test that’s designed to test an allele burden of 0.4%. My test came back as “not detected” my heam says PCR tests are highly accurate & they are designed to find small amounts of cancer cells that might be missed by other types of tests. You are correct in saying PV JAK2 negative is very rare on top of a already rare disease. He said he would treat me with PV if I was JAK2 negative and if my EPO was low. I would also like to think that it is probably a good thing that my platelets actually lowered after a phlebotomy to 360 and then after 4 weeks went down to 315 because platelets usually go up after a phlebotomy with this mutation. However, I will add that depending on the laboratory values, everything was in the normal range on my cancer center app as apposed to my pcp offices from my first blood draw

[hunter5582 in reply to Healthy6386]

You are correct that PLT usually goes up after a phlebotomy. PLT can vary by as much as 100K in a single day in response to what is going in in your body.

It sounds like you have a good understanding of what is going on. As your hematologist suggests, it seems unlikely that you have PV or some other MPN. Best still to follow up and be sure you know what is the status/cause of the erythrocytosis.

Do please let us know what you learn and how you get on.

[Healthy6386 in reply to hunter5582]

Do platelets only continuously increase with phlebotomy if you have the JAK mutation or is that because of the process of repeated phlebotomies. Just trying to figure out if it’s a good that my platelets went down after a phlebotomy after a recheck in 4 weeks & then went down even further on their own

[hunter5582]

Phlebotomies can produce a short-term reactive thrombocytosis. The issue with repeated phlebotomies is chronic iron deficiency. The purpose of repeated phlebotomies to treat PV is to induce chronic iron deficiency to control the erythrocytosis. The body must have iron available to make RBCs. Most of the iron used to make RBCs is recycled from expired RBCs. Removing RBCs removes iron from the body. While therapeutic phlebotomy is used to reduce the risks associated with erythrocytosis, the iron deficiency comes with its own potential adverse effects.

Your platelet variation is within the normal range. The fact that it is not trending upwards towards PLT > 450 (diagnostic for thrombocytosis) is a good thing.

Hope that answers your question.

[Healthy6386 in reply to hunter5582]

Thanks again. My heam who has treated a few MPN’s said it was a good thing that my RBC’s were 4.8 and hematocrit at 43% after a phlebotomy. said with PV your red count would still be elevated while your hematocrit in range. Cause your body is constantly producing RBC’s unless I was on some type of medication

[hunter5582 in reply to Healthy6386]

With the the JAK2 mutation, the "on switch" is always "on" - making too many blood cells. It can be RBCs, PLTs, WBCs, or any combination of all three. It also causes the overproduction of inflammatory cytokines. It is an interesting condition, like the old Chinse curse "May you have an interesting life."

I hope you get your situation figured out and make things very boring (from a medical perspective).