Hi everyone! I’m new here and have a few questions. My husband is 26 years old and was recently diagnosed with primary thrombocytosis after a visit to the ER. His blood test results came back with him having a platelet count of 1 million. He’s being seen at a military hospital and was referred to internal medicine where his doctor prescribed him aspirin to take while waiting for an appointment with the hematologist. When he was seen by the hematologist he was told to take hydroxyurea. Before taking it my husband continued to try and ask if he could just continue taking aspirin but he was told that it may cause him to bleed out so the doctor told him to start taking hydroxy which has lowered his counts to 800k but it caused him to feel tired all the time. He was told to stop hydroxy and doctor prescribes anagrelide one pill twice daily. We were never told of any mutations and I’m actually still kind of confused about all these different mutations all we know right now is that my husband has a high platelet count (right now at 900k) and that he has primary thrombocytosis which they don’t know what is causing it. My husband is trying to get a referral for a civilian doctor for a second opinion but we haven’t been successful with that so I was wondering if any of you could help just kind of give me an idea of what my husband should be asking his doctor or what it is we should do? I have been reading of some scary side effects that anagrelide has caused some people so I was wondering whether or not he should continue taking anagrelide or should he ask to be put back on hydroxyurea? They also said he has an enlarged spleen but doctor doesn’t seem too concerned about his spleen. I’m concerned about him having to take these meds as I have seen some people say they currently are not taking medication for it and refusing hydroxyurea and other meds. Sorry for a lengthy post but thank you in advance!
High platelet count? Anagrelide? Hydroxyurea? - MPN Voice
High platelet count? Anagrelide? Hydroxyurea?
First, I’m very sorry to hear about your husband. He is very young to have to suffer with this disease. It’s always scary to start taking hydroxy but that seems to be the standard protocol. I personally couldn’t handle the side effects of anagrelide but everyone is different. My advice is to stay on the medication. His platelets are still very high and the enlarged spleen is a concern. I’ve been dealing with ET for over 11 years and my medication has had to be adjusted many times. I’m glad you are getting a second opinion.
Let us know how things are going. You will find a lot of support on this site.
Thank you! My husband did alright with the hydroxy except just feeling tired all the time I was looking into Anagrelide and the side effects of it just sounds a whole lot scarier so it’s very concerning.
Four thoughts. A proper diagnosis of essential thrombocythemia requires a bone marrow biopsy. Hydroxyurea will cause infertility in the man and birth defects in his wife. You should have been warned to use barrier contraception or abstain. In many places a 26 year old would be treated with aspirin and interferon. We are talking about very rare diseases and you are right to seek a second opinion. This should be from an MPN specialist.
When my husband first saw his hematologist the hematologist told him that he wouldn’t need a bone marrow biopsy unless he wanted one. We were told that it can cause birth defects and that when we were going to start trying for another to let his doctor know so he can be put on something else. Is the interferon that you’re talking about the pegasys drug?
Hi, it would appear that your husbands haematologist has categorised your husband ‘high risk’ for some reason, hence treatment.
At the young age of 26, he would normally be considered ‘low risk’ - unless he has had platelets above 1.5 million, a previous thrombotic event, or is particularly symptomatic. You say that your husband had a visit to the ER where his high platelet count was discovered; was that visit in relation to anything that the haematologist may have based his decision on?
I think that your husband should get a second opinion due to his young age, preferably with an MPN expert. You should clarify with your Dr which criteria was used to diagnose your husband with ET. For example, have they tested for mutations such as Jak2, CalR, or MPL, sometimes you can be ‘triple negative.’ They should have informed you of this. It’s a complex enough condition to understand without being left in the dark.
Interestingly, the 2016 WHO (world health organisation) reclassification of ET was updated to include a bone marrow biopsy as a major criteria for diagnosis.
Hi! My husband has not had a thrombotic event. When he went to the ER he thought he had the flu & he previously had pneumonia years ago so he went in to get checked out and blood tests results came back showing that he had high platelet counts so the doctors sent in a referral for him to see the hematologist. My husband just told me that his most recent visit his hematologist told him that he has the CalR mutation. Also, they said that he wouldn’t be doing the bone marrow biopsy unless my husband wanted it done.
Although no mutation is great, CalR is the ‘better’ one to have. In ET, thrombotic events are more linked with having the Jak 2 mutation.
The haematologist is right to be cautious with aspirin and the risk of bleeds with ‘high’ platelets; however, it’s usually considered ‘risky’ at around 1.5 million.
Have you looked on the MPN Voice UK website? There is lots of up to date, trustworthy information on MPNs there. Also, Patient Power, USA, which has a section called ‘Ask the Experts.’ I found it very helpful when I was diagnosed with ET. Educate yourself as much as possible and be your husbands best advocate.
It would be good if you could get a second opinion from an MPN expert if possible with regards to a discussion on ‘is any treatment necessary?’
I actually just took a look at that website thank you for that info! Very helpful. Sadly there’s no MPN specialists around here I tried searching for one (we are stationed in El Paso) but we’ll look at our options to try and go to another city. I have been wondering that whether or not treatment is even necessary right now we will discuss this with his hematologist. Thank you so much!
Yes, MPN Specialists are few and far between. There are two renowned MPN Specialists in your State of Texas; Serdan Verstovsek, MD Anderson and Reuben Mesa, San Antonio. However, I realise Texas is a huge State and there is some distance involved.
Good Luck
Thank you for the doctor recommendations! I will definitely look into them and figure out how to get him a referral to an MPN specialist! And thank you! We live 15 minutes away from where the shooting took place but we are safe thank you!
Thoughts are with you. Just heard of the shootings in El Paso.
Pegasys is a long acting form of interferon. From what you post an MPN specialist might propose not treating your husband at all but rather monitoring his condition. He has CALR, is asymptomatic, with platelets well below 1500. You can get more information at this link: nccn.org/patients/guideline...
Pay special attention to the section on essential thrombocythenia.
Thank you! From what I’ve been reading on the posts here a lot of people don’t take hydroxy right away or any other meds but get monitored that’s why I was always confused why my husband was put on meds right away without any thrombotic events or symptoms besides the high platelets.
Please don't be too concerned over the side effects of hydroxy and Anagrelide, my wife has been taking three Anagrelide a day and one hydroxy every other day for six months now. Previous to that she was on differing amounts in a bid to keep her platelets in line. Her spleen was removed 14 months ago after it ruptured partly due to being enormous! And a fall helped!. Her platelets were around 1.8 million too. Now her platelets are around 275,000 and all other blood results are normal. I think that once they get the platelets down to normal you will find the tiredness will go away. Keep an eye on your husband's hemaglobin as low iron will cause tiredness. If it is low ask for an iron infusion, they work wonders😃. And all the best for both your futures. PS ask for copies of all blood tests, and keep a record of all meds and results.
That’s great to hear that your wife is doing good! I hope she continues to do well! I will keep all this in mind thank you! My husband’s platelets reaches 1.3 million and when he started hydroxy it went down to 800,000 but is now going back up. We’re really concerned about the anagrelide side effects that’s why I think we’re gonna try and see what other options we may have. Hopefully we can quickly get a referral off base to see an MPN specialist.
I agree in having a bone marrow biopsy for proper diagnosis. Hope you'll find a right specialist soon.
Thank you! We will talk to his hematologist about that because he was initially given the option not to get the biopsy done.
I live in Florida and would highly recommend dr Verstovsek at MD Anderson in Houston. I travel to see him every six months just to follow up for a second opinion. Should you have any further questions feel free to message me directly. I am ET jak2 positive.
Thank you!
Unfortunately your husbands experience is all too common for people with MPNs. These disorders are so rare (1/300,00) that most docs 9even hematologists) lack the KSAs to provide optimal treatment. No matter what it takes, be sure to consult with a doc who really does have the needed expertise. I found one on this list of patient recommended MPN expert docs, mpnforum.com/list-hem./ .
It sounds like he is getting on the rights track re. his diagnosis. With the blood tests and knowing he is CALR positive, the diagnosis of ET can be made without a bone marrow biopsy. Many docs have moved away from doing these routinely. The main reason to do one would be to look for evidence of fibrosis.
Regarding the meds, sorry to hear about his experience with HU. While some are able to tolerate this med and benefit from it, not all can. I am also HU-intolerant. You should have been told that HU is teratogenic, mutagenic, and carcinogenic. It suppresses the immune system and need to avoid live vaccines. In men HU can cause hypogonadism (oligospermia/azoospermia) and there is some research indicting it may cause decreased testosterone as well. HU does pass into semen, so he should have been told to use a condom to protect his partner. HU is so teratogenic that women of child-bearing years are advised to not even handle the bottle without gloves. Since he is off the HU - this is all water under the bridge; however, having been on it he will need to wait one year before having any children. Many docs will not use HU in younger patients due to all of the above. Hope the anagrelide works better for him.
Regarding the aspirin and bleeding risk - that is something that needs close evaluation by a doc who understands MPNs. For many people with thrombocytosis, the risk is for thrombosis and aspirin is protective. However, some people react differently and actually have an increased risk for hemorrhage. It used to be thought that this only occurred at levels well above 1 million, but recent research has shown this can occur at levels well below 1 million. The learn more about this, look into Acquired von Willebrand Disease. I suspect there are some of us MPNers who may fall short of full blown AvWBD, but still have some level of altered platelet function in the direction of bleeding. When my platelet levels get up above 700K, my von Willebrand Factors tend to drop below normal levels, but I do not appear to have full AvWBD. FYI - lots of hematologists are not up-to-date on this. I had to look up the testing protocol for the AvWBD assessment - give it to my doc - then find a lab that could do all the tests indicated. The MPN Specialist is saw (not my regular hematologist) took me off aspirin as he felt my risk for hemorrhage was greater than my risk for thrombosis. This is NOT a standard recommendation for age>60 JAK2+PV.
Hope all of the above helps. The best thing your husband can do would be to do whatever it takes to see a true MPN Specialist. I have to travel to see the one I picked - totally worth the time and expense. All the best to both you and your husband. FYI - the good news is that this is a condition that can be managed. I was originally diagnosed with ET over 30 years ago. Still alive and kicking!
Thank you so much for taking your time to respond to this! I greatly appreciate it.
The only side effect my husband was experiencing was fatigue but that could also be due to the heat (we’re in El Paso & he works out in the motor pool all day) so I was always telling him to keep hydrated but he went ahead and talked to his hematologist and he decided to switch his medication to anagrelide but after reading some of the side effects we started to worry on whether or not he should continue taking anagrelide or go back to hydroxy. He’s going to go back and talk to his hematologist again. I knew hydroxy isn’t safe for pregnancy and we were told to tell his hematologist if we were going to start trying again but do you know if anagrelide have the same effect? I think his hematologist mentioned interferon for when we decide to try for another baby. My husband was also taken off aspirin previously he was told to just take hydroxy (his hematologist was concerned he would bleed out due to his platelets being 1.3 million at the time)
Anyway thank you again!! We will definitely be trying to get a referral to see an MPN specialist.
All of the chemotherapies come with potential adverse effects and we all react differently to each. There is some good research on the use of peg-interferon for MPNs, but it also comes with its own unique set of risks and benefits.
Here are the particulars on Adverse Reactions for anagrelide from ePocrates.com.
Serious Reactions
= CHF
•arrhythmias
•torsades de pointes
•ventricular tachycardia
•hemorrhage
•stroke
•thrombocytopenia
•interstitial lung dz
•interstitial nephritis
•pulmonary HTN
•hepatotoxicity
Common Reactions
=headache
•palpitations
•diarrhea
•asthenia
•edema
•nausea
•abdominal pain
•dizziness
•pain
•dyspnea
•flatulence
•vomiting
•fever
•rash/urticaria
•chest pain
•anorexia
•tachycardia
•pharyngitis
•malaise
•cough
•paresthesia
•back pain
•pruritus
•dyspepsia
•photosensitivity
If you do not n have an account with ePocrates - the basic access on-line is free. You can access the Professional level drug monograph, patient education leaflet, and drug interactions check. epocrates.com/
Regarding the splenomegaly - that is something to monitor. If the doctor is not concerned about it then it is likely only mild enlargement. This is common in MPNs where the spleen is overtaxed in dealing with too many platelets. That does not mean it should be ignored - definitely stay aware of the issue when the platelet levels are so high. I have had mild splenomegaly for many years - sometimes a bit larger than others - it fluctuates. The docs stay aware of it, but do not consider it a significant issue.
There is lots to learn for you and your husband regarding his MPN. I have not looked into the CALR mutation much as I am JAK2+ originally ET that progressed into PV. Here is one journal article you may find of interest bloodjournal.org/content/12... .
All the best on this journey.
My opinion is he should ask for a BMB to see what is really happening and define his illness. First thing he should do before anything else due to his enlarged spleen.
I was diagnosed with PVR way back in 1983 and was given a drug named Busulphan and 4 pints of blood was taken over two or three weeks and then I was put on hydroxy and was on it, in varying doses to control all the bloods, from then until 2013. It caused me to have mouth ulcers for most of that time, and red and sometimes lumps on my face, which was Rosacea, but most of the time the bloods were controlled. Platelets did rise to over 880k and I was put on Analgrelide but blood tests showed the creatinine increased, so I was taken off this. A few months later I was tried on Anakgrelide again, but the creatinine went higher and stayed there. so I was told not to go on them again. Creatinine is a kidney related measure, so I had to attend the kidney specialist! Anyway, the creatine is stable at the increased level and is being measured each time I have a blood test, which is every 3 months at this period in my life.
Fatigue can be due tu the hydroxy or the blood condition.
I had a bone marrow biopsy in 1983 which proved the Polycythaemia and again in 2010 when my blood changed to MF. This change was recognised by the regular blood tests. I have been on Ruxolitinib since January 2014 and straight away the mouth ulcers stopped, the redness in the face stopped and the spleen went into hiding.
Just to let you know that the bone marrow biopsies were not too painful, but for me, a bit uncomfortable for a few days afterwards, and I cannot remember taking painkillers after.
I am nearly 73 and still enjoying life, travelling, skiing, motorcycling, and lots of voluntary work.
I hope you and your husband can have a good long and fulfilling life.
Michael
Hydroxi didn’t work for me so in addition I’m on anagrelide. I’m confused. I’m u see the impression he should also be on asprin.
It’s a really difficult time for you. Your husband is young.
I wish you well.
When my wife was on Anagrelide and her platelets were about a million after being on HU for about 10 years. With her oncologists approval she took tocotrienol from red palm oil. Slowly it reduced the platelets and her doctor adjusted the Anagrelide frequency as the platelets came down to the normal range.
Is there anyway I can lower my platelet count?
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