I am a 74 year old living in the U.S. - diagnosed with ET CALR 2 mutation in 2021. Was put on Hydrea aka hydroxyurea and low dose aspirin immediately. After about a year, began developing side effects and decided to stop the hydrea - against my doctor's recommendation. No, she is not an MPN specialist. Went for almost a year with no medication at all. I am otherwise very healthy and active and never had symptoms from the ET. However, my platelets quickly went up to one million five hundred. Got a second opinion (still no MPN specialist - another well respected hematologist/oncologist), plus a bone marrow biopsy and additional blood tests. Resulted in same diagnosis. Now on Agrylin...just started. I don't understand why this is not the first course of action. Is there a problem with Agrylin? What experience have any of you had with it?
From the research I have done, the CALR mutation makes me less susceptible to clotting ..it is my age alone that requires I take a medication to lower the platelets.
Any information you can share will be appreciated.
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mpnunlocked
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Hello and welcome. It sounds like you have done your research and have a good base of knowledge. You are correct that people with CALR are less prone to thrombosis.
You would likely fall into the intermediate risk level in the commonly used risk stratification system. The recommendation is to use cytoreduction for most people in this risk level. Note that this is a recommendation not a requirement. There is room for individual variation on the treatment decision based on the person's individual MPN profile. The guidelines about cytoreduction apply to large number of people and may not always be the most appropriate choice for an individual. That is why it is so important to see a MPN Specialist who has the depth of experience to know when it is OK to depart from the standard guidelines.
The other factor in your decision is that it is based on your treatment goals, risk tolerance, and treatment preference. Your doctor can make recommendations but you make the decisions. If cytoreduction meets your criteria for use, then that is the most important factor. The next step would be to determine which agent you would prefer to use.
Hydroxyurea is a widely used first-line treatment for ET. While it is used off-label for MPNs, it is in the NCCN guidlines for MPNs. Anagrelide is FDA approved for ET but is considered a second line treatment option due to a more severe adverse effect profile. Note that we are all different in how we respond to these medications. Some people cannot tolerate HU but do OK on anagrelide. There are other options as well. Pegasys is also used to treat ET. There are also ET clinical trials underway for Besremi and Bomedemstat. You do have options if you decide cytoreduction is indicated.
Like you, I am HU-intolerant and I was refractory to it for treating PV with erythrocytosis and thrombocytosis. I have done much better on the interferons, Pegasys and Besremi. The IFNs have been very effective and the side effects have been minimal. That is my experience. yours may be different.
If you continue to have questions about your treatment plan, suggest that you consult a MPN Specialist for answers. The decision may be no different but your understanding and level of confidence would likely be improved. Here are two lists of MPN expert docs.
Thank you for the thorough response. I will try the anagrelide for a while and see how it works. My second opinion Dr. said if I could get my platelets below one million by taking a minimal dose that it would be sufficient. Both doctors advised against getting a colonoscopy at this time as my platelets are so high it could cause bleeding. My sister had colon cancer - so I feel I should have this test. Anyone else been advised against a colonoscopy?
The short answer is that you should get a colonoscopy, but not until your risk of hemorrhage is acceptable. If you have PLT about 1 million, your are likely at greater risk for hemorrhage than thrombosis. Cytoreduction is indicated in this situation.
With a family history of colon cancer and having a MPN, you are at increased risk for colon and other cancers. You definitely should be following up on a colonoscopy. Until you can have one, suggest reviewing the non-invasive tests like Cologuard or a virtual colonoscopy CT or a colon capsule endoscopy. Your gastroenterologist can give the best guidance on what would be a better option for you.
You are fortunate to be getting good guidance from your MPN care team. They will be able to advise you when it is safe to move forward with a surgical procedure and can consult with any surgical team you need to access.
I was put on Hydroxycarbomide about 6 years ago to treat my ET but at the time I didn't tolerate it well and so after 6 months was put on the second line treatment Anagrelide (I also have pulmonary sarcoidosis so was put on Methotrexate and steroids at the samw time which probably contributed to the nausea).
Anagrelide is not as effective at controlling platelets and even after increasing the dose my platelets were still erratic. So after 5+ years with no apparent side effects my haematologist said that they may need to change me back to Hydroxycarbomide or Interferon and was told that once you reach 60 the drug would need to be changed anyway - I'm 58. In order to see what was going on I had a bone marrow biopsy in June that sadly revealed that I have Grade 2 fibrosis. I was advised to go back on Hydroxycarbomide (I didn't really want to go on Interferon).
I was told that the fibrosis could be just part of the ET or in some cases can be caused by the Anagrelide??
Have to say that the Hydroxycarbomide side effects aren't as bad as I remember so am hoping it was just all the other meds I was taking at the same time.
Anagrelide is great and I would have loved to have stayed on it but things are progressing so that's why I'm on Hydroxycarbomide the first line treatment.
Can I ask why you are so hesitant to try an interferon?
I'm always curious, but it has been shown to reduce the progression of MPNs aa well as transformation to AML- which neither HU nor Anagrelide have.
RE: your question marks about anagrelide being associated with progression of/to myelofibrosis:
"A study by Campbell and colleagues suggested that anagrelide induced a significantly greater increase in bone marrow reticulin than did hydroxyurea as well as a higher rate of transformation to myelofibrosis at 5 years.214 Interestingly, four patients who developed fibrosis while taking anagrelide showed regression when switched to hydroxyurea. These findings suggest that consideration should be given to performing surveillance bone marrow biopsies every 2 to 3 years in patients receiving anagrelide treatment."
from:
Thrombocytosis- Jamie Koprivnikar MD, Craig M. Kessler MD, MACP, in Consultative Hemostasis and Thrombosis (Third Edition), 2013- Anagrelide Therapy
I was hydroxy for ET for 3 years then developed a reaction to it, was changed to Anagrelide which lowered my platelets, I had a few palpitations on it at first but they settled down. I was on it with no problem for quite a few years. I’ve progressed to MF now and am on peg interferon.
Hi - I have been diagnosed with ET Calr for 30 years now, (I am 57) was on hydroxyurea initially for about 4 years, then quite some time on aspirin only and my platelets were around 1,500 but I was much younger. However I was advised to go onto Anagredlide when I was 44 and I have been on this with aspirin for 13 years (take 3 per day) and it has worked very well for me - no side effects to note. Hope everything goes well for you with your change of meds.
Forgive delay in reply mpnunlocked. Living in England, I have little option than to stay on Hydroxycarbamide. Afraid I do not know Agrylin ... but please do keep reporting how you get on with it. Do you still take aspirin along Agrylin?
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