I have been treated with Hydrea 500mg a day since January of 2021. My bone biopsy that year indicated ET/Jak2. I am seen at a local hematologist here in Alabama but I go to MD Anderson to see an MPN specialist annually.
My visit this year did not go well as my hematocrit % has steadily climbed over 3 years. MDA looked at my bone biopsy that was done here locally and still concluded it was ET, however, my MPN Specialist thinks I have PV without this data supporting it.
I have kidney disease, renal stenosis, gastric issues, high blood pressure, and high cholesterol.
MY MPN specialist is recommending a phlebotomy every 3 months if my hematocrit number goes above 45.
Has anyone progressed from ET to PV? I see that Jak2 occurs in 90% of PV patients. My allele burden is average at 17%.
Any insight is appreciated.
Thank you!
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Auggie17
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Hi. I started Hydreasin two years ago one capsule a day. Last year it was found I had renal stenosis out of the blue. I suffered much pain and in two days I am having surgery. I read somewhere that Hydroxy does that. Any idea about it? What about your case? I am thinking of changing to Besremi as I have enlarged spleen and liver and I also have saturation.
I have not heard that about renal stenosis but it is a symptom of PV I believe. I hope the Besremi helps with your issues as I have heard good results with others that are on it. Good luck! If you are getting a stent put in to your renal artery, it’s a very simple straightforward procedure.
"Polycythaemia vera represents a rare chronic myeloproliferative neoplasm characterized by an increased thrombotic risk. Previous case reports have documented a link between primary or secondary polycythemia and the presence of renal artery stenosis and renovascular hypertension."
BTW, I frequent either printout and hand articles to my MPN MD, or else email them through the patient portal.
It may help that I'm a PA, but they always say that they appreciate the information, and it helps me explain qhy I may have a certain concern or philosophy.- oh, and always seem to have read and thought about the articles.
I was diagnosed with ET over 30 years ago. It progressed to PV about 11 years ago. This progression can and does happen but it is not an instantaneous process. It is more of a gradual change. I am wondering how long ago your BMB was done. It may be that things have changed since then.
For a female with HCT > 48% and JAK2 positive, it would be reasonable to be thinking about a PV diagnosis. If the diagnosis is confirmed, maintaining HCT < 45% becomes the treatment priority. This is more important than PLT levels in regards to risk of thrombosis. Note that some MPN Specialists use HCT < 42/43% for females.
You may want to review your treatment options with your MPN care team. The other treatment options for PV would be Besremi (or Pegasys) and Jakafi. Besremi has demonstrated efficacy in terms of progression free survival and reducing JAK2 allele burden. Jakafi may have this same benefit. This is not something that HU can offer as a treatment outcome. All three medications can do an effective job controlling blood cell numbers. Sometimes phlebotomies are needed to supplement cytoreductive medications to keep the doses at a tolerable level.
Thank you Hunter for your response and valuable information. My BMB was actually in 2020, and I agree that it could have changed since then. I do think my MPN specialist believes I have PV and will see him again in April 2025 unless something changes here with my hematologist. At least now I know what I am dealing with and can advocate for myself a bit better given this information. I will definitely speak to my MPN specialist about changing my treatment options.
Yes it’s known to happen. From what I’ve read from firsthand accounts transformations tend to happen mostly to those who are older 55+ which is consistent with study results. I second getting on besremi given your age and emerging transformation.
I progressed from ET to PV jak2+. In my case little changed blood count wise. More likely a different opinion. Many of us probably on the border of ET/PV. My treatment didn't change. I was already trying venesections with HCT at 49% to help with headaches and fatigue. Likely the real world is more fluid than rigid HCT targets. Some of suffer the symptoms before meeting the criteria.
ET to PV is pretty common, keeping your Hct below 45 or some say 43 for female is essential with PV to reduce thrombotic risk. A AB of 17% is low which is probably (but not proven) a good thing.
Hi, I was diagnosed with Jak2+ ET in 2017-18. I was having blood tests every 3 months. During the pandemic I missed one set of tests and when I went back 6 months later and had tests I had progressed to PV with HCT over 50% for 3 months straight. My MPN specialist told me at that time that about 15% of diagnosed ET will progress to PV. Just my experience, we are all different. Best to you.
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