Is ET a blood cancer?

[Growingforward]

Hi everyone. I am new to this support group and been following you for a couple of weeks now. I am very impressed with all the comments and help offered. I was just diagnosed with ET a month ago and started on Hu after my bone marrow biopsy. I am now up to two 500 capsules a day and it has definitely brought down my platelets count. I have two questions I would like to put out there. First, my hematologist says he does not consider this blood disease a cancer although everything I read says it is a rare blood cancer. Second, I have had weak, splitting and very thin nails for years. Could that and also a crawly itching feeling in the abdomen be symptoms of ET?

[hunter5582]

Welcome to the forum.

ET and the other MPNs were reclassified as blood cancers by the WHO in 2008. Not everyone agrees with this and some still prefer to call them "disorders. " My own take is to call ET/PV cancer with a little "c" not Cancer with a big "C". You are more likely to die with ET than from it. There can be very real challenges in managing ET, but they can be met.

The thin/splitting nails can happen for a variety of reasons, including iron deficiency. The HU may make this worse, but does not always do so. There are a variety of reasons for the abdominal symptoms. Some can be related to the ET.

MPNs are at the core inflammatory disorders. The dysregulation of the JAK-STAT pathway causes the overproduction of inflammatory cytokines. This is thought to be responsible for many of the secondary symptoms we experience.

It is very important to consult with a MPN Specialist regarding your treatment for ET. Most hematologists do not have the KSAs to provide optimal treatment due to how rare MPNs are. Here is a list of MPN expert docs.

World-wide mpnforum.com/list-hem./

USA pvreporter.com/mpn-speciali...

Cytoreductive therapy for ET is indicated when you fall into a high-risk group. I believe that you noted some discomfort with taking hydroxyurea. It is one of the two standard first-line treatments for ET. The other first-line treatment is PEGylated Interferon. Each med has its own risk/benefit profile. We each respond differently to each of these meds. Your hematologist should have already reviewed each of your treatment choices with you. Here are a couple of articles on this topic.

mpnjournal.org/how-i-treat-...

legeforeningen.no/contentas...

Hope this helps

[Growingforward in reply to hunter5582]

Thank you hunter5582 for your reply. My doctor seems to really know what he is talking about and I was told people come from all over to see him. But not sure if he is considered an MPN specialist. So far other than fatigue and digestive issues my side effects are pretty mild. Just hate the thought of taking a cancer drug for life. I have other issues such as high blood pressure, high cholesterol, osteoporosis, neuropathy, insomnia and I am pushing 75 so guess I am high risk. So thankful I found this forum and can hear from people going through the same thing. Blessings to you and all who share.

[Gullyfolly in reply to hunter5582]

This is a very very helpful post, thank you. I am an athletic 49-yr old recently diagnosed with ET. My platelet levels reached 1,200 a couple months ago and we’re working them down with HU. As I work with elderly friends & family with various flavors of cancer this helps put it in perspective. My hematologist (who I love!) refers to ET as a sort of pre-leukemia rare blood disorder, but I don’t feel that she is avoiding facts so much as managing my outlook. In a weird twist, my high platelet counts were first detected during a routine blood test after a heart attack last year. Turns out I don’t have heart disease but rather had blood clots due to ET. Good news-ish!

[Growingforward in reply to Gullyfolly]

Very interesting Gullyfolly. We are all certainly on our own journey with this "condition". I also have heart disease and my cardiologist has me on five different meds. Be great if HU was the answer to that too. Staying positive with you. Attitude really is very important in how we respond to our health issues.

[hunter5582 in reply to Gullyfolly]

I can go with any of the various descriptions. Some consider the MPNs to be in the class of leukemias. That would be leukemia with a little "l" not a big "L". At the end of the day it really does not matter. What matters is how our MPN presents and the actual impact it is having on our lives. MPNs are all over the map in terms of the symptoms and severity. Best to just take it one case at a time and deal with it as it actually is.

Glad to hear you have a hematologist who you love. I actually have two. I have a MPN Specialist who is about 2 hours away. Great doc, but too far to go for routine treatments needs. I see him semi-annually. I have local hematologist who handles my on-going care. I absolutely love this doc! He actually listens to me, answer questions, and respects my right to make my own choices. He even spends more than 15 minutes!!

My docs acknowledge that PV is a cancer, but we just do not focus on it because it does not matter. What does matter is how my MPN is presenting, progressing, and interacting with my other medical conditions. (I have more than a few). We review treatment options then I make treatment decisions that both docs respect. It is the ideal arrangement for a less than ideal situation.

ALl the best to you.

[Growingforward in reply to hunter5582]

I agree with you that it doesn’t really matter. Cancer or not to control the platelets we still have to take the chemo pill. My platelets were actually in the normal range today for the first time. But he said I will still have to take two a day to keep it there. Hopefully with time I will start feeling better. Thanks once again for your input.

[hunter5582 in reply to Growingforward]

You may actually find that once things stabilize you can back off a bit on the HU. Do note that there is no particular value to platelets being under 450 for most people with ET. It is about controlling symptoms - how the blood cells behave - not how many platelets there are. There are many MPN Specialists who look at treatment targets this way. When a numeric for platelets is used, 600 is the target recommended by some of the leading MPN experts. silvermpncenter.weill.corne...

onclive.com/view/treating-p...

legeforeningen.no/contentas...

Having said that, treatment targets need to be individualized based on your specific profile. Some people do find they feel better and experience less symptoms when their platelets are "normal." You have significant other medical issues that need to be considered. The benefits of HU need to be balanced against its toxicity. The same is true for all of out treatment options.

Wishing you all the best and continued success.

[jevans59 in reply to hunter5582]

Thx for pointing this out. What other treatment targets are important to watch?

[hunter5582 in reply to jevans59]

In general for all of us with thrombocytosis as a component of our MPN the primary risks are thrombosis, hemorrhage, and microvascular events. The absence of the symptoms is the primary goal of treatment. Docs tend to focus on thrombosis risk most since it tends to have to most serious consequences. However, hemorrhagic event scan also be serious as well as microvascular events.

For people with PV it is actually erythrocytosis and leukocytosis that create the greatest risk of thrombosis. That is why HCT is used as the target for treatment. The evidence is quite clear that risk is decreased when the erythrocytosis is under control.

The other goals/symptoms to monitor are more case specific. Fatigue, pruritis, hypertension, insomnia, erythromelalgia and other common MPN secondary symptoms are primary treatment targets. The management of these symptoms is a primary treatment target.

Do lets be clear that many people with MPNs do need cytoreductive treatment. Anti-platelet treatment is also often needed. The consequences of not treating adequately can be very serious. As several in this thread stated, thrombotic events can cause heart attacks. What each of us needs to do is work with our care team to establish treatment goals based on our individual case profile. We have to weight the putative benefits of the goals against the intrinsic risk of the treatment. Hydroxyurea is a "highly toxic medication with a low therapeutic index." PEGylated interferons carry significant risks as well. All of our treatment options do.

The other type of treatment target is for the treatment chosen to not involve unacceptable risks or cause significant adverse effects. This is nearly always dose-dependent. When doses are increased to achieve a treatment target, risks are increased as well. There needs to be clear evidence that the benefits of achieving a treatment target outweigh the risks of the treatment needed to achieve the target.

The point of the above referenced literature is that there is no particular value to platelet levels being <450. There is no linear relationship between platelet levels and risk of thrombosis.

In my view the best approach is to work with a MPN Specialist to set treatment goals based on each patient's unique presentation of the MPN, the patient's treatment goals and priorities, and what risks the patient is willing to tolerate. The treatment goals need to be based on evidence-based protocols of reduced risk, not trying to make labs look "normal."

Hope that answers you question.

[jevans59 in reply to hunter5582]

Thanks, Hunter, I have an appointment next week and will ask what additional targets in addition to the platelet count will my Dr be watching. Thx again!

[Runner999 in reply to hunter5582]

I don't know about that ! I have no symptoms of ET at all but my MPN clinic will put my hydroxy up if I go above 500 on platelets.

I am on it because I am mid 60s and have peripheral arterial disease - so was already stented in the thigh and taking clopidogrel when rising platelets discovered on "well woman" check ups.

[hunter5582 in reply to Runner999]

That is understandable as you have multiple risk factors. That is the individualized part of MPN care. The 600 number cited by Dr Silver is one recommended goal, but should not necessarily be used or all patients. One of the benefits of HU is not just that it reduces the number of platelets but also the risk of microvascular events. How the blood cells behave is actually more important than an absolute number. With peripheral artery disease this would be a particular concern.

Wishing you all the best a success in managing the ET.

[GVW-Sydney in reply to Gullyfolly]

I am in the same boat as you Gullyfolly. Three years ago when I was 45 years old I went for my regular 40km bike ride and a few hours later was picked up next to the road after a heart attack and cardiac arrest. Blessed to be alive and thankfully a guy who passed by saw me and started CPR early enough, ambulance arrived and restarted my heart and got me to a hospital in time to remove the thrombosis from the artery. As I was very fit and healthy and not know what casused it, I kept on searching for the cause of the blood clotting. Eventually, two years later I was diagnosed with ET. At least I know now what caused it and it can be managed. At first I was on Pegasys, which my body did not like. Now I am on HU and it works wonders. Hapy days! Great to be alive!!!

[Gullyfolly in reply to GVW-Sydney]

Wow that’s incredible on so many levels GVW-Sydney! Did you suddenly fall from your bike? Sounds like in a rural area? Yikes! Mine was on my road bike in the countryside but was so mild I had no idea it was heart related and coasted on, had some water, and continued home without stopping. It wasn’t until I felt similar symptoms playing pickleball a week later (a week of biking, pickleball, canoeing, and badminton btw) that I went to a doctor. They quickly diagnosed the heart attacks (now thought to be one, two-part episode) based on troponin and detected high platelet but didn’t say anything about them. ~9 months later I had chest pain which turned out to be from repetitive stress from playing a VR game 🤣 and they detected that my platelet count had risen further to and investigated. At least now I feel that every blood measure is being monitored, including by me directly via the MyChart app. Take care and bike on!!

[Oceanviewer2019]

Perhaps your hematologist only considers it a precursor to cancer? I was diagnosed with ET in Aug. 2017. I have seen 3 hematologist since that time and all of them consider ET a blood cancer. You may consider seeing a hematologist who specialized in blood cancers.

[Growingforward in reply to Oceanviewer2019]

Thanks Oceanviewer2019. This doctor works at a cCare cancer center and is highly respected. He said unless there is a tumor he doesn’t consider it a cancer. But he is treating me with a cancer drug. So treatment probably wouldn’t change if I changed doctors. He did say that with long term use the HU could cause leukemia. This is all quite a learning process.

[paradiastole in reply to Growingforward]

The evidence linking leukaemia with hydroxycarbamide is quite tenuous (it's actually used to treat some types of leukaemia) - and if there needs to be a tumour for something to be called a cancer, what is leukaemia? It's best not to get hung up on the c-word, though. Look through social media and you'll find people panicking because they have just been told they have "incurable blood cancer", and that's not really the best way to think about MPNs.

[Growingforward in reply to paradiastole]

That was my question too paradiastole. My first reaction was pretty negative when he said I needed to go on a chemotherapy drug. Maybe that is why he is soft-peddling the blood cancer issue. No matter, it is what it is and the Hu seems to be doing its job. I am just taking one day at a time. I live alone since losing two husbands within three years time so coping the best I can. I don’t feel so alone on this now that I have found you all. God Bless.

[hunter5582 in reply to Growingforward]

You are not alone. We are one big MPN family here. We don't always agree, but we do always care. That is what families do.

All the best my MPN sister!

[Growingforward in reply to hunter5582]

Hunter thank you so much for the added information. It helps me a lot. I may have to get a second opinion here pretty soon. As much as I like my doctor, he wants to keep me on 2 pills a day no matter what my platelets level. I will give it a little more time, then insist on going to one a day or ask for a second opinion. So happy to be part of this caring MPN family.

[hunter5582 in reply to Growingforward]

Getting a second opinion is a standard of medical care and every patient's fundamental right. Good docs never mind a patient getting a second opinion. Good docs also welcome input from a colleague with greater expertise in rare disorders. I would not wait. MPN Specialist are pretty rare and it can take several months to get an appointment. If you are anywhere near Baltimore Maryland, the docs at Johns Hopkins MPN Center are terrific.

All the best.

[Growingforward in reply to hunter5582]

I am in California so guess I better start doing some research. Thanks again for all your suggestions and advice. Bless you.

[hunter5582 in reply to Growingforward]

MPN Specialist lists

World-wide list mpnforum.com/list-hem./

USA only list pvreporter.com/mpn-speciali...

[Mostew]

I tend to not get hung up on the diagnosis Cancer . It is what it is .. aAs Hunter says little c

Concentrate on what you can do to feel better .

This group is so helpful and stops us feeling alone x

[Growingforward in reply to Mostew]

Thanks for your reply Mostew. I agree and I think that is exactly how this doctor feels. He doesn’t want me to get hung up on the Cancer classification. So glad I discovered this forum.

[Lifam]

Welcome to the group. Yes, ET is blood cancer but as Hunter said we're more likely die with it instead of from it. Best to find yourself a haematologist who is specialised in blood cancers(MPN specialist). Did you mean you're having 500mg HU daily, not 500 capsules daily I hope.

[Growingforward in reply to Lifam]

Oops. Guess I didn’t phrase that very well. I am taking two 500 mg capsules a day and it has brought my platelets down to a normal level of 338,000 in one months time. I see him again in two weeks.

[Minu68 in reply to Growingforward]

I was startled on 1 capsule a day, then they raised it slowly, to now taking 2 on 3 days of the week and 1 on the others.My haematologist said I’ll stay on this dose for now, as it’s keeping my platelets at acceptable levels, but that it will depend on my blood tests, and might be changed up or down accordingly.

Sounds like your treatment has been far more ‘aggressive’ than mine, they took 4 months to bring mine down. I also take aspirin, as many of us do.

I think your doctors certainty about not changing the dosage ever should be questioned, as mine say it takes time to find the right dosage and that it ideally is the lowest amount possible where chemo meds are concerned

Welcome to our group, stay safe!

[Growingforward in reply to Minu68]

Thank you Minus68. When I asked him about lowering my dose now that I am back to a normal range, he just said he has no one on just one a day unless first starting out. But we would watch it for awhile and it is possible to adjust it down later. At least he said never say never. I definitely will insist on lowering it at some point because I feel the same as you. I will not take more of this drug than I absolutely have to. I am also taking the aspirin.

[Gullyfolly in reply to Lifam]

I’m up to 1,500mg HU daily now and my platelet levels are finally getting down to the 500k zone. I hate taking so much since HU itself is a carcinogen, but it seems like the best course.

[Mpn1967]

ET is a bloodcancer...

[ladyanello]

American health/ life insurance companies consider it a cancer so maybe your doctor could explain that it isn't to them!

[azaelea]

Welcome Growing forward! I hope you’re managing alright and the comments and advice on here are helping. I too have ET JAK2 diagnosed 3 years ago when I was 82. I’m on 500 mg Hydroxycarbamide and Clopidogerel . I also have various other issues such as high blood pressure ( controlled ) and diverticular disease so a lot of us seem to be on multiple medication unfortunately. Hoping you keep feeling upbeat and this friendly forum helps with any worries you have. Regards,Fran

[Growingforward in reply to azaelea]

Hi Fran. Thank you for your welcome. It looks like we all have a lot in common. Definitely good to have all this support. One day at a time. 😊

[Wyebird]

Welcome, the cause of splitting nails is something people have mentioned but not me. Although I swear by argon oil for dry skin hair and nails. Yes cheap too.

[Growingforward in reply to Wyebird]

Thank you Wyebird. I will pick up some argon oil.

[Wyebird]

It’s a cancer, and if he argues is he really in the know about MPN’s?