Polycythemia Vera Management Often Does Not Follow Guidelines

[Manouche]

« Overall, the authors said their data suggest physicians are overreliant on phlebotomy, even though a relatively low percentage of patients are achieving recommended HCT control and a significant number of patients are experiencing thrombotic events »

ajmc.com/view/polycythemia-...

[jon1972]

Thanks for the post Manouche. I've been wondering about whether I should be keeping my HCT even lower than 45. My doc at Cleveland Clinic seems to think it's OK if I drift above 45 whereas I've been thinking I should never be hitting 45 to begin with as I had a heart attack and stent placed several years ago, I believe as a result of the PV. I was pretty young to be having a heart attack (mid 40's), but the cardiologist who placed the stent said I had CAD. Other cardiologists I've had since say that doesn't make any sense since I have no family history or risk factors for CAD, and that it's more likely the PV is what caused my heart attack. So this is all kind of supportive evidence. I've heard other folks on here say that their doctors keep their HCT below 44 or even 43, but my current MPN specialist disagrees.

[EPguy in reply to jon1972]

The HCT studies found a rapid rise in risk right at the 45 cut-off. Suspiciously cleanly divisible by 5 but it was their known result. So one with a prior cardio risk may benefit from staying under 45 with only brief exceptions. But if the common info is the reference, being far below 45 is of decreasing benefit. A guy at 43-44 avg with 45 max, as you say, does seem about right to me.

Cleveland is a top cancer center so it would be good to understand their risk assessment in more detail.

[ainslie in reply to jon1972]

It is very concerning your current haem is saying that, for any male 45 Hct is max and no harm in keeping well below that especially with your history. The thrombotic risk rises exponentially above 45 and all the guidelines for PV say less than 45 for males and some say 42/43 female. In your case 1000% I would say keep it under 45 max and if in doubt get a second opinion, if your haem thinks over 45 is okay for you I would look for another haem anyway as what he is okaying is dangeroes for anyone with PV and more so for you. Cleveland Clinic is good generally I think but maybe not with MPN, maybe worth seeking a MPN specialist even if its only to get direction and consult from time to time, they can usually write to your local haem if necessary. Dont mean to scare anyone by repeating the exponential curve thing but better safe than sorry.

[Manouche in reply to ainslie]

 »The thrombotic risk rises exponentially above 45 and all the guidelines for PV say less than 45 »

Ainslie, you keep mixing up the exponential level of blood viscosity above 45, with a so-called « exponential » risk of thrombosis. The increased risk level of thrombosis is not to be confused with the exponential increase of blood viscosity alone. In other words, the risk of thrombosis is increased with a high HCT, but is NOT exponential.

[ainslie in reply to Manouche]

I’m not getting mixed up, it’s the same thing in reality

[Manouche in reply to ainslie]

 »While there may be a relationship between higher hematocrit levels and an increased risk of thrombosis, it is not necessarily an exponential relationship.Studies have shown that blood viscosity increases with higher hematocrit levels, which can potentially increase the risk of thrombosis. However, the relationship between hematocrit levels and thrombosis risk is complex and may be influenced by other factors.Furthermore, the relationship between blood viscosity and thrombosis risk is not straightforward either. While it is generally accepted that higher viscosity increases the risk of thrombosis, this relationship is also influenced by other factors such as blood flow rate, shear stress, and platelet function.Therefore, it is not accurate to assume an exponential increase in thrombosis risk with increasing hematocrit levels. It is important to understand that thrombosis is a multifactorial condition, and many different factors can contribute to its development »

[ainslie in reply to Manouche]

Can I ask you who wrote that and in what article or publication and when. Its contrary to everything I have read or heard on the subject, the most recent a presentation by Dr Spivak at the Doc to Doc conference. For others reading this who are not so interested in the fine print the message is keep the Hct below 45 in males and if poss 42/43 in females

[Manouche in reply to ainslie]

This is a pretty standard text on this topic written here by ChatGPT. I don’t think you’ll find an haematologist saying something different.

And we all agree about the 45HCT upper limit for men.

[ainslie in reply to Manouche]

Chat GPT

ChatGPT is an AI chatbot that uses natural language processing to create humanlike conversational dialogue. The language model can respond to questions and compose various written content, including articles, social media posts, essays, code and emails.!

[Steve_Essex in reply to ainslie]

we probs don’t want to be going down an AI rabbit hole but ChatGPT can process and assimilate 1000s of publicly available articles on the internet and summarise based on the context of the question asked… so there is some legitimacy in using as a source to understand complex topics.

Personally I have no issue in people here using as a tool especially as scientific journals and topics of this nature can be extremely difficult to break down and understand..

however I would caution using as an authoritative source of information as it currently has no ethics or morals - it doesn’t (at the moment) discriminate between well respected and less credible research…. And could unintentionally, or intentionally be the source of misinformation

[ainslie in reply to Steve_Essex]

well said, I think its better we follow the Haem experts instead

[Meatloaf9 in reply to jon1972]

Hi, may I ask who your doc is at cleveland clinic? I am seeing Dr Gerds at cleveland clinic.

So far my HCT stays around 44 on HU. I think he prefers to used meds and not phlebotomy to control HCT.

[jon1972 in reply to Meatloaf9]

I've been avoiding naming names, but I have Dr Gerds as well. I've had issues with one of his nurses getting in the way and speaking on his behalf which has been more the problem than anything. I won't use the messaging system anymore because she is the one responding and I don't trust her. That said I still have a high opinion of Gerds, but I think he gets caught in the middle with this highly opinionated nurse.

[Meatloaf9 in reply to jon1972]

In my opinion, he should have total control in the information dispensed by his nurse. I think I am also starting to see a problem with a person who responds to messaging. I don't think a nurse should be making decisions for a mpn specialist. I have also had a problem with the people who schedule the return visits when leaving, their system of signing in and waiting for them to call you in to schedule a return visit is absurd. Last month they made me wait about 30 minutes just to schedule my return visit while I watched them doing nothing. I did sound off on the post visit questionnaire sent to me by Cleveland Clinic. We'll see if it does any good. I do like Gerds. I hope he gets the clinical trial he wants.

[jon1972 in reply to Meatloaf9]

Is that the one for that HCT reducing drug? I've really been struggling with IFN keeping my HCT counts in range and Gerds brought up a drug for that during my last video visit.

[EPguy]

The study goes from 2011 to 2019. In 2011 Rux did not exist as a PV option and IFN was mostly limited to Dr Silver's group and selected Euro practices. But they suggest less reliance on Plb and more on other options, including HU.

"the most common therapeutic approach, phlebotomy alone, was associated with the lowest level of guideline-compliant results (14%) of all the therapeutic pathways"

They don't break it down finer by year. But we see right here on the forum that practice has/is changing rapidly toward the end of that period and esp since 2019. Further in (a quick look at) the detailed study, IFN and Rux are buried in the tiny "other" box without even a mention, see sample plot here. HU is reasonably represented, but probably could be used more.

We now know here that Plb is not a great long term solution esp if frequent. Drs should be getting this message.

My guess is if this study were for example from 2020-2023 the result would have a much heavier representation of the more modern solutions.

But the broad idea shown stands, all pts should be offered the latest options when appropriate rather than be limited to only the older methods.

Plb in PV

[monarch5000]

It was an MD Anderson/Texas study and that cancer center is so behind the times that as of Dec. 2022 they were still prescribing hydroxyurea as a first line treatment for PV. Proof: youtu.be/leCa6ZwZ5k8(Not working as expected?)

The Silver MPN center/New York, by contrast, recognizes the #1 cause of premature death, 12 years after a PV diagnosis, is progression to myelofibrosis, not from blood clots. So the Silver Center prescribes interferon at the time of initial diagnosis, instead of hydroxyurea, because in their 35 years of clinical experience, interferon much more reliably enables PV patients avoid progression to myelofibrosis.

[Dan73 in reply to monarch5000]

I have PV. I was told by a doctor at the Silver Center at Weill Cornell that at my age (mid 70’s) hydroxyurea would be best to control blood counts. 500mg/day does the job. I have been taking it for 2 years with no problems. I asked about interferon and was told that at my age the side effects outweighed the benefits.

[havashan in reply to Dan73]

I am 80 years old. I was diagnosed with an MPN (probably ET) and was advised to start Pegasus as the only drug that could possibly slow progression of the disease. I started on a low dose 25 ML every other week which has slowly lowered my platelet court to 325. I have no side effects from the drug and I have very little if any symptoms of the disease. So I do not think that someone in their 70s should be concerned about using this treatment. I started seeing an MPN specialist at Johns Hopkins who started me on Pegasys. When he left JH I started seeing an MPN specialist at Weill/Cornell who agreed with my treatment. Don't let old age stop you from trying an effective treatment.

[ainslie]

Manouche

I am not sure this makea any sense ie your wrote

"The Silver MPN center/New York, by contrast, recognizes the #1 cause of premature death, 12 years after a PV diagnosis, is progression to myelofibrosis, not from blood clots.

According to a recent presentation by Gisslinger the average progression from PV to MF is 6% at 15 years, so its highly unlikely at 12 years after PV diag the number one cause of death is progression bearing mind they would have to progress to MF and then die all within 12 years. I would speculate that the rate of death from PV progression at 12 is almost non existant. If I recall 30% of PV deaths is from thrombosis, confirmed at a consult with Dr V.

And I dont think many would agree that MD Anderson is behind the times....., as a patient there thats certainly not my experience over the last 6 years

[mfh7 in reply to ainslie]

I agree with you. I am a PV Patient at MD Anderson and was prescribed besremi as a low risk patient and told to keep my hct below 45 as a male both of which appear to be consistent with the modern wisdom on the subjects. My prior doc had me doing phlebotomy for the prior 5 years since diagnosis and I was severely iron deficient with incredibly high platelets. I am extremely glad I moved to Dr V at MD Anderson who I believe has me on the right course. As I think we discussed it is unfortunate that he recently retired. Having said that, treatment methods for PV are patient dependent and somewhat fluid as new studies come out regarding the benefits of early drug intervention so we must all educate ourselves regarding the new studies.

[Manouche]

Hi ainslie, I’m not the author of the post you’re referring to, you’re referring to a post written by Monarch50000.