Interesting article re PV treatment options - MPN Voice
Interesting article re PV treatment options
Thanks Paul, very interesting and easy to read. I am a stronger proponent of Peg-interferon than the author is, but many good points are made in the article. I was surprised he does not prescribe aspirin in many cases: for me aspirin has been very effective as it has stopped visual migraines and indeed migraines that I had on a regular basis.
Thank you Paul. Interesting to get another view. Kindest regards Aime x😻
Dr. Spivak is the consulting MPN specialist who works with me and my ongoing local hematologist regarding my care for PV. The protocol reflected in this interview is the treatment protocol I am following. Dr. Spivak provided me with a full copy of the article he published in the July 2019 Blood Journal, which goes into more of the underpinning research behind his treatment recommendations. His actual treatment recommendations are more varied based on the profile of the patient than the interview might suggest. What he does not do is just put everyone on hydroxyurea + aspirin (or any other medication). The use of medications are to alleviate symptoms when needed as part of the patient's treatment protocol. He does in fact use aspirin and medications to reduce blood-cell counts when they are warranted based on a patient-specific risk-benefit analysis. Like most docs treating PV, we does recommend phlebotomy to control HCT.
There are other treatment protocols out there which take a different approach. I am a strong believer that one-size-does-NOT-fit all. Too many docs follow a "standard protocol" without truly looking at the individual needs of the patient. Some docs are not staying up-to-date on MPN treatment because they see so few MPN cases.
As you may be aware the American Heart Association recently changed its age-based recommendation for the routine use of aspirin to prevent cardiac issues. It turns out that our body's response to aspirin changes as we age. Some of us are at more risk for hemorrhage due to the aspirin solely based on age. However, some people who are symptomatic due to PV do in fact need to take the aspirin - but not all.
In my case, when Dr. Spivak saw me and evaluated my case he recommended against the aspirin. I am age 64 and have a history of excessive bleeding and bruising while on aspirin and it was getting steadily worse. I also have a history of von Willebrand factors that tend to drop below norm when my platelet levels get into the 700-800Ks. Dr. Spivak rather prophetically said "you really do not want to get a brain bleed." Two weeks after seeing him, I was diagnosed with a hemorrhagic brain tumor. I had bled in my brain. Aspirin was not such a good thing for me.
I do encourage everyone to review the different treatment options and to realize there is more than one way of thinking about how to treat PV or any other MPN. Dr. Spivak's approach is to focus on reducing symptoms and extending life span, but not to focus on "sanitizing" the numbers on a CBC that may not affect actual risks or symptoms. This is the right approach for me. It may not be the right approach for others. I am pleased with the phlebotomy-only protocol I am following and am comfortable with the inherent risks/benefits of this approach. I hope everyone finds an approach with which they are comfortable and successful in managing their PV.
Thanks for that insight.
To what extent is Dr Spivak focussed on trying to prevent disease progression? The INF bulls claim that like all cancers, MPNs should be treated as early as possible. INF is the only ‘front line’ drug that targets the HSCs and can therefore, for some, impact progression.
Did he have any thoughts on the recently announced long term INV v HU v Venesection trial?
I note that he does advocate INFs for high risk PV but how is high risk defined? % JAK2AB, fibrosis, excessive proliferation, symptom burden, additional mutations, age , physical health. Be interesting to know how he ranks these factors.
Aspirin is a dilemma. Any established standard procedure pressurises all Hems to continue with it until clear weight of clinical evidence proves otherwise. Is this what we are seeing with HU v Pegasys.
Best Paul
Here is a quote from the paper he presented July 2019 Blood Journal.
" PV treatment has 2 goals: alleviating symptoms and prolonging survival through prevention of thrombosis, intractable splenomegaly, and leukemic transformation. Adequate phlebotomy therapy alleviates symptoms due to hyperviscosity but not severe migraine, aquagenic pruritus, or erythromelalgia. Control of thrombocytosis may be necessary for migraine relief or TIA, for which I prefer pegIFN to anagrelide; aquagenic pruritus has many therapeutic options depending on its severity: ruxolitinib, pegIFN, psoralen and ultraviolet A,97 and hydroxyurea. Erythromelalgia classically responds to aspirin.98 Thrombocytosis induced von Willebrand syndrome usually does not cause spontaneous bleeding; for minor surgery or dental procedures, tranexamic acid or e-aminocaproic acid suffice; for major surgery, platelet count reduction to achieve normal ristocetin cofactor activity is necessary. "
Dr. Spivak's focus in actual treatment is dealing with symptoms and prolonging survival. He tends toward peg-interferon when chemotherapy is needed, but does not favor hydroxyurea generally speaking. He goes into some detail about this in the paper. The next time I see him, I am going to ask about slowing disease progression. if I need chemo again, I would lean towards peg-interferon based on the research I have reviewed. It appears there is some disagreement about the value of molecular remission, but I connote help but to think it would be a good thing.
All the best.
When I was dx mid 2016 my AB was 56%. By March 2018 it was 80%. That prompted me to start Pegasys.
Got my Dec AB result last week - down to 13%!
This despite also having the TET2 mutation which is meant to impair Peg efficacy.
What is the long term significance?
By itself, rising AB does not correlate 100% with progression but my understanding is that it is a risk factor and may increase odds of additional mutations
But is AB reduction a possible marker that Pegasys might be working at a deeper level and for some, this does impact disease progression? ie can you have Peg induced improving fibrosis if AB still rising?
And, bottom line, whilst Pegasys does appear to delay progression for a subset of users, does this significantly extend lifespan? Intuitively you would think yes but the INF v HU v Venesection trial implied not...?
Best Paul
Amazing to read you’re down from 80 to 13% in 2 years. You could well get a complete molecular remission in the next few years. It’s hard to image this would not extend dramatically your lifespan if not curing your condition!
If you are interested in Dr. Spivak's whole article I have permission to share it. if interested, message me with your email and I can send it to you.
Hi Paul, I was Dr Spivak patient until he started only doing one day in clinic and as he thought trying interferon would not hurt I switched to his partner Dr Moliterno since I can see her more often. Dr Spivak is a fabulous Dr, he has studied this disease longer then anyone I think and continues to do research. He is a believer that medicine is only needed for certain things and that we each are so very different! He also believes there is a indolent and aggressive part to this disease and is a wise and gentle Doctor. He has published many articles in the New England Journal of Medicine. Every Doctor that I even mention his name says he is a Great Doctor! My local hem who is not a fan if his says he wrote the book on MPN's. In that clinic they have about 30 people on Pegasus some have done extremely well and others have not. He is not a fan of HU and is not too much a fan of aspirin unless really needed.
Thank you for the article
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