I was diagnosed with ET three months ago and as I am high-risk (over 60, JAK-2 positive, another mutation and a couple of TIA episodes) I have been prescribed daily aspirin and weekly Peg-interferon.
I seem to have responded well to the interferon, as my platelets have come down from over 800 to around 500, after only six doses.
I have read that there is no direct correlation between high platelet counts and stroke risk and I wondered if any of you could explain that to me. If this is so, why is my consultant so keen to get my platelet reading to normal levels? Does this mean that even if I achieve that, I will still be at high risk of having a stroke?
Thank you in advance!
Sarah
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Ginevra
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I'm Steve from Sydney... These days, I am Post ET / MF... (64yo). However, I am renowned for having an extremely high platelet regime, which is also convoluted by another condition, (Von Willebrands Syndrome - VWS), which simply means that one moment my Blood has too much clotting protein, and in the next... not enough...
My 'DRIVER' is also different to yours as I am CALR (2)... My Platelets are usually stable between 700-800s... However, whenever they have been well in excess of those levels, (in the past), I had possibly THREE (3) TIAs, (minor brain strokes).
Hence, while my diagnosis is somewhat differing to your own, in my mind... Higher Platelets have ALWAYS been evident whenever a TIA developed, in my view...
Couple of other 'Red Flags' for me are: Vision issues; Tinnitus & Headaches!
These days, I must resort to taking Methotrexate (MTX), to prevent that occurring... Don't like taking MTX. However, it manages my abberant Platelets when I require assitance etc.
I no longer take (enteric-coated Aspirin), as Clopidogrel seems to work better for me...
Hope something in my reply might aid your enquiry...
Hi all I too have ET Jak 2 apparently for some years but diagnosed a year ago .
Over those years I can now see from blood results as my platelets rose my visual disturbances headaches and some balance issues also
. I was investigated a couple of times for stroke after high BP and loss of speech but as no apparent evidence of clot or bleed nothing was done until the visual problems along with many other and varied symptoms were almost daily .
My Gp referred me back to stroke unit … TIA has never been confirmed but severe changes to white matter and an area called the Putamen were found .
When started on clopidogrel the symptoms from the visual and headache went . But eventually a BMB showed ET .
also have Sjogrens and Antiphospholipid syndrome and on Hydroxychloroquine and Hydroxycarbamide … my platelets at their highest were 750 nothing compared to many in this site but obviously had quite an effect on me .they reduced very quickly and remain on low end of normal . I have fatigue still but not the frightening visuals and speech loss though brain fog and burning feet at times is distressing . I guess i am agreeing with Steve’s very succinct thoughts . But equally find the thought that the correlation between platelets and clots is unproven i also have the antiphispholipid problem which also increases risks.if clots . so it is quite a minefield of complicated symptoms and from all the people i keep meeting is much more common than it would seem . Lots of luck L
Thank you all for your replies, which have given me a lot to think about. You all make very useful points. I'm going to need an energetic day to try to understand the Haematology article! In my case I think aspirin is helping, because since I started it I haven't had any ocular migraines and these were occurring several times a week previously. I also had one episode where I went completely blind in one eye for about 15 minutes and fortunately that hasn't happened since starting treatment.
I think what you have all got across to me is that this is a complicated and unpredictable condition where there are no simple answers. I shall continue to read and learn! Best wishes to you all and thank you for taking the trouble to respond.
I see in your prior posts you have a hard time getting a formal Sjogren's Dx. This is common, it can take some pts years to get one. But as you know Sjo can cause just about every symptom there is, and make itself known after years of being mild in the background.
If you at some point consider IFN, read the post "My Last Dose" for some ideas for a safe journey.
Thank you I read your post with interest and frustration on your behalf.
I have realised having these conditions come with built in frustrations which for our sanity we learn to deal with . My first rheumatologist dismissed my Sjögren’s blood test and all other symptoms and told me to distract myself by working harder.. it is reassuring when others in this site share their experiences and I am grateful for that .
Reading papers on Sjogrens recently I suspect I had had it for many many years and the suggestion that it made me much more likely to develop MPN have you read anything along those lines . My sister has RA and also recently had a stroke . I do wonder about familial autoimmune conditions.
quite by chance , I came across this paper yesterday. I haven’t yet had a chance to read it fully- just a quick glance , but the title initerested me as I also have high platelets/ ET/ Jak2 + ..but no history of clots /TIAs . My haem was always pushing aspirin for clots, but the research papers I read said otherwise. So I checked with an MPN specialist- 2nd opinion - and she totally agreed with me and the regime that I use, ie not what the regular haem said.
“Aspirin in essential thrombocythemia. For whom? What formulation? What regimen?”
It’s from : Haematologica.org
Of course, ET is rare, but strokes/ TIAs etc are not. The vast majority of people who have these have platelets in the normal range . When I worked as a physio on stroke rehab, I never came across anyone with raised platelets, let alone that being the cause of their problems . There are clearly other factors at play here, most likely related to diet and other risk factors.
You have asked a rather complex question that is a common one for people with thrombocytosis. It is true that there is no linear correlation between the absolute number of platelets and risk of thrombosis. There is, however, a correlation with risk of hemorrhage when platelet levels are higher.
The risk of thrombosis has more to do with how the blood cells behave than how many of them there are. Bear in mind that platelets comprise less than 1% of your total blood volume even when the numbers are elevated. One explanation is the "extra-sticky" theory, that the blood cells become more prone to adhering to each other and the vascular endothelium. Many MPN Specialists now believe that there is no clinical benefit from making the number of platelets "normal." There is no evidence to support his goal. Some MPN experts use 600 when an absolute number is used. Others consider the delta (degree of change) to be more important.
The risk of hemorrhage increases with the level of thrombocytosis. This has to do with alteration in von Willebrand factors that are needed for coagulation to occur. If the thrombocytosis is too severe (usually over 1 million) then there is risk of developing Acquired von Willebrand Disease, which is a bleeding disorder. Someone with thrombocytosis can exhibit increased hemorrhage risk without the AcqvWBD. This used to happen to me when my platelets went above 800.
MPNs are rare disorders. Most doctors, including hematologists, have little experience with them. Not all hematologists are up to date on the current thinking that drives treatment for ET and the other MPNs. That is why consultation with a MPN Specialist is so important.
Given your history, there would be no question that cytoreductive treatment is indicated. The issue is what the platelet target should be. Reaching an absolute number of PLT<450 may not be a relevant goal. The goal is the prevention of thrombosis, microvascular symptoms and hemorrhage. In addition, treating any secondary or constitutional symptoms you experience is also very important. Prevention of disease progression is another important target.
Prevention of inappropriate platelet aggregation and microvascular symptoms would sound like an appropriate treatment target, particularly in light of a history of TIAs. The question is what do you need to do to achieve this goal. This is a discussion best to have with a MPN Specialist who can do the best job of guiding you through the risk/benefit analysis related to your medications, dosing, and ultimate treatment approach. Here is a list of MPN expert doctors. mpnforum.com/list-hem./
Thank you, Hunter, for your detailed and helpful reply. I don't think I had appreciated the seeming paradox of increased risk of both bleeding and clotting.
I am very fortunate in that I live in Oxford, so am under the care of the haematology team at the Churchill Hospital.
I am JAK 2 + I was on Aspirin and Venesections, 1st diagnosed about 9 years ago. When I was diagnosed my platelets were 1143 with a HCT of 0.614.
I was put on a fairly high dose of Hydroxy, eventually I was asked to go on to Pega and reduce my hydroxy, Pega took about 3 years to bring my numbers under control, so after 6 Pega you are doing well. I did have over 66 venesections.
My allele burden, has dropped to about 2%.
My current numbers are ok, but they did drop my dose from 135 to 90 and my numbers went up slightly.
Pega does affect my sleep and one tip, try not to inject in the same spot, that caused me a slight skin rash.
Thank you for your reply. All those venesections sound alarming. I hope you mean that now you are on Peg you no longer have to have them? After hearing your experience I am even more surprised and pleased that my platelet count has come down rapidly. Thank you also for your tips. I am being careful to inject in a different area each week. I had not realised that Peg can cause insomnia, but have noticed that I sleep particularly badly the night after I have had my injection. All best wishes.
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