I had a clinic appointment last week and when I got to the hospital, discovered that my usual haem was unfortunately on leave for 6 months. They’d therefore drafted a replacement in whom I saw, who immediately recommended I go on hydroxy or Interferon.
Now, my usual haem has seen me for 4 years since diagnosis, and taking into consideration my age (48) and the fact she sees me as low risk, has been happy to continue with aspirin and venesections. I have PV, am jak + and I have raised platelets which have been around the 750 since diagnosis. I also have high WBC count.
So I’m a bit confused - it seems that test results are open to interpretation and opinions can be divided as to the best way forward. I must admit it came as a bit of shock as it was not what I was expecting, so haem sent me away for 2 weeks to think about it.
I’m really not sure what to do. There seems to be a school of thought for younger patients to hold off as long as possible but another, to start on Interferon as soon as possible, which I am considering.
I was just wondering if anyone else has found themselves in a similar position? And if so which way did you go?
Many thanks
Pete
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PT99
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Hi I am 41 diagnosed in December with ET/PV awaiting BMB results. My platelets were very high at initial diagnosis so I went on treatment straight away, the 1st doctor put me on Hydroxy which I had no problems with but after further investigation and advice from people here I changed to interferon due to my age. I also have a high WBC count. I was told venesection wasn't an option as my platelets would rise further. I am also on asprin.
I await my next haematology appointment Friday to see how my blood counts are doing on interferon. I think it's a very difficult and a scary decision but I just took the advice of my consultant and this forum for further advice. The support here has been invaluable. All the best.
I am on Roferon A Interferon generally it's been ok. My platelets at starting treatment were >1000. Hopefully treatment is starting to work after appointment on Friday. Good luck and let me know how you get on.
I had a similar decision albeit I was in my early 50s. I was content to go onto HU because a) of the reduced risk of stroke/thrombosis b) there's some evidence that HU protects against fibrosis c) studies of HU don't seem to suggest an increased risk of progression/cancer, at least over a couple of decades
but the younger u r I guess the more c) weighs in your decision
I am in a similar situation but I’m very symptomatic and leaning toward asking for interferon. I am afraid at my age (almost 40) to start on HU. I have potentially 4-5 decades left and that’s a very long time to be on HU. The 10 and 20 year studies don’t quite reassure me.
I’m older than you and was diagnosed in 2009 and treated with venisections and aspirin. I switched hems last year and put on HU. It stopped the itch and lowered the platelets ( from 976 to the 300s). My first and second doctors disagreed about my treatment. I went to a third who prescribed HU- now I wonder if that was the right hung to do.
On November 9, 2017 I went to a patient symposium at Weill Cornell NYC last year and heard every PV expert there say that they did not prescribe HU for a number of reasons most importantly because it does not address the underlying issue. Instead they prescribe Interferon which has the potential of knocking out the clone if administered early enough. That may be your new hem’s reasoning.
I wish I had been given that option from the beginning. I would suggest that you do some investigating on your own. Check out Dr Hans Hasselbalch -Copenhagen; Dr Richard Silver- Weill Cornell who have written about why they have taken this position. Also Dr Ruben Mesa - Mayo Clinic, Arizona and University OfTexas -and Dr Gotlieb at John Hopkins (think). They’ve all taken the same position.
You can watch much of the Patient Symposium. It’s online . I don’t know if the PV session was recorded- I hope it was.
I know many doctors do not start treatment unless the patient is symptomatic and/or in their 60s. That may be your original doctor’s thinking.
The MPNs are a complicated group of blood disorders There’s a lot to know and treatment is a tricky process. My humble opinion is that the best thing you can do is to learn all your options and the reasons behind each and then find an expert hem to work with. I do hope this is helpful.
I had exactly the same situation, diagnosed at 42, 1st consultant first put me on anagrelide whoch didn't really make an impact on platelet count then just aspirin & venesection after I started getting heart palpitations. Platelets started creeping up and I felt more & more tired but consultant said I could get to 1500. New consultant comes along, refers me for a bone marrow & keen to put me on hydroxy. He leaves but next consultant agrees and puts me on hydroxy. It has been 9 months and tiredness much better and platelets now 250. Only issue is that my haematocrit keeps going up so had to restart venesections.
At the time of diagnosis, my haematocrit and haemagloben were a little raised too. I was put on hydrea which lowered my platelets, haematocrit and haemagloben.
I’m in similar position but age 62, PV/MPNU with aspirin/venesections. Being offered HU/Peg thanks to my age but fit, in good condition and therefore Hems relaxed.
My dilemma is whether early low dose Peg will reduce progression risk. Amazingly even the most renowned expert Hems around the world are split opinion.
There seem to be three camps
Drs Silver and Hasselbalch are gung-ho start asap, best chance of slowing progression risk. They have been prescribing INF as first line c. 20 years.
Drs Verstovsek and Yacoub more circumspect . INF could reduce progression but they only have five years Clinical Data. Hopeful but need more data.
Drs Tefferi and Vannucchi recently published article in Blood Journal based on available data. They believe HU still first line choice, INF/Peg second. They are concerned about long term side effects of INF. Claim no additional risk of MF/Leukaemia with HU. There is also a question mark over definition of ‘slowing progression’.
My opinion:
INF/Peg does appear to reduce the JAK2 Allele Burden (% of mutant JAK) and in some cases can significantly reduce fibrosis. However there is a degree of uncertainty over whether this alters actual progression risk into MF or is more ‘cosmetic’, ie there is something else going on. Some Hems believe that current research into JAK 2 is going down wrong path.
Other factors to consider
HU appears to be faster acting, hence good choice if need a immediate reduction from dangerously high counts
HU better tolerated than Inf/Peg which has 20-30% early drop out rate. Peg can cause thyroid issues and depression. Hence those prescribed high dose may be better off with HU.
Recent year two trial results for Ropeginterferon versus HU had HU ahead at 6 months, level at 12 months and Ropeg ahead at two years with significantly reduced Allele Burden FWIW!
Too sum up, confusing mixed messages! I sympathise with our Hems as they try to balance multiple issues such as quality of life, long term risks, side effects and ease of use versus the possibility that long term trials of Peg will prove it does slows progression, albeit perhaps only for a small % of patients treated.
My intention is to start low dose Peg within next few months and hope to switch to Ropeg when available. I will also be keeping an eye on RG7388 Phase 2 trials which I think are starting in USA.
Thanks for your reply. I too have read up on the opinions of Drs Silver and Hasselbalch, which has made me think Interferon is the way to go mainly because of the chance of slowing down progression.
My new haem’s concern is that she should be doing what is required to lessen the chance of clots/strokes, as, if something did happen, she wouldn’t be doing what is right by me. I don’t suppose any of us really know what the risk is but apart from the PV, I’ve always eaten healthily and kept fit, which I still do.
If the professionals are split on certain drugs/treatments, it’s no wonder patients find it all a bit confusing and I don’t mind admitting, a tad overwhelming in my case. It’s obviously not straight forward and there’s no ‘one size fits all’ treatment.
I have annual medicals so can track my Platelets rising from about 2012/3. I was dx ET (after BMB) Sept 2016 by local Hem/Lab but this was revised to Masked PV by Guys in Oct as my RBC/WBC/HCT started to rise. Been on venesections and aspirin. Like you, eat well and keep fit so everyone okay with me staying off drugs despite age (62) risk.
Had BMB in Dec, two cores taken. Local Hem said ET, Guys say PV or MPN - Unclassified. My blood counts still imply early PV with RBC just above normal, WBC a fraction below. Venesections every two - three months.
What is your WBC? Very high WBC increases thrombosis risk. And your Allele Burden?
My Allele Burden was 60% last check which does predispose me to progression but more an additional risk factor than a given. As I posted yesterday, there is some debate over the significance of AB but my guess is more in relation to other factors (some still unknown) that cause progression.
Whatever, better to have low AB than high AB. I think the averages are, very roughly and from memory, around 25% ET, 45% PV and 70% MF. This is not something to fixate on though, just read a post on MPN-NET from someone with PV over 10 years and 95% AB.
Having said all that, it is the principle reason I intent to start Peg in the near future. In the interests of balance, I should stress that everything I’ve read about Tefferi, who is cautious Peg but +ve HU, indicates he is a lovely guy, brilliant and totally dedicated. If he is steering his patients away from Peg to HU, he has good reasons.
I was diagnosed with ET in 2003 and been on salospir 100 since then. My platelets are between 550-650. No venesections. ( I have very low iron and normal spleen) My haematologist is a professor. During the past years my haematocrit has risen and now it is 51,8. (On the limit as they say). As I am a woman it is higher than should be but still he keeps me with the aspirin. Other doctors that I saw during all these years told me terrified that I should start Hydroxyurea immediately!! Fortunately I did not listen to them. Recently I had a second bone marrow test and it says that I have PV. I will see my doctor to discuss it. I do not exercise at all and have gained weight so I plan to start very soon. I also plan to buy a bike. I tell myself that when I start exercising and loosing weight my haem. will drop. Don’t know if this is true. I dread medicines also reading in here of the side effects!! I am 67 years old but look and feel much younger. I try not to eat a lot of red meat. I will keep you posted if anything changes. Be well!
PS. I spoke with my haem tonight. He told me since my platelets are lower than 1000000 I should not worry. Also since my spleen is not enlarged I can exercise. But he suggested that I start Hydroxyurea 1 tablet a day and see what happens. I don’t know if it will also lower my haematocrit.
It’s good to be given this choice. I’m 50. I’ve read every paper on PV and meds. There is a medical evidence that high WBC counts increase the patients risk factors. Also if you look at what was presented at Decembers ASH conference. You’ll see that interferon has been proven to be superior (long term). I’m happy being on pegasys. At the end of the day. Choice is good, when the patient has all the information. My information is what I understand. It’s up to you to read the information and come to your own decision.
Pete, tongue ulcers. Which have had to be removed surgically (not 100% sure it’s directly linked to interferon). First couple of injections I had mild flu like symptoms, during the night. A lot more positives than negatives.
Thanks so much for all your replies. This forum is an invaluable resource and it’s wonderful so many want to share their stories and take the time to do so.
There is much food for thought here and I’m still weighing up the options but I’m seeing the haem again tomorrow to discuss further. I’ll post an update in due course.
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