I had 1.5 mil platelets and now they are 645K

[stai]

I am new here. Great board, great posts, great people.13 Years ago (2003) my platelets progressed to 1.5 mil in about a year. Dr. suspected ET but couldn't confirm, bone marrow test was inconclusive, as he said. He suggested hydrea, I refused. I even don't take a baby aspirin as told because it gives me bad gastro problems. I do have all the symptoms of ET, but I carry on with the average trouble. The Hem sent me for many tests to look for secondary cause of high platelets, nothing found. The platelets were moving between 1.2 and 1.5 mil. After about 10 years, I went for surgery for BIG benign polyp. I lost blood and was given one batch of transfusion. Since then, my platelets were gradually coming down by about 50K per month and after 13 years with ET, they are now 645K. I still have all the symptoms of ET. In 6 months a new Hem (old retired) will see me the second time, and the JAK2 and CALR tests will be available. This is my only second testing, first was bone marrow test. Anybody had similar experience where platelets gradually go very high and then down to just little high? My theory: The polyp was malign, but the microscope/instruments could not catch it. It was causing a secondary thrombocytosis? I am worried they might go too low transforming ET into AML.

[SilverET]

Hi Stai,

My understanding is that this can be a natural progression of ET and I had that same thing happen. My platelets at diagnosis were well over 1mill but as I was so young and still growing the Drs decided not to treat with anything (not even aspirin). I have had my clots and issues but generally coped quite well. Over the years my platelet numbers dropped to just above normal levels. I was infomed that as some of my bone marrow is normal and some carries the mutation it can depend on what clones of megakayocytes (cells in the bone marrow responsible for producing platelets) are active at any time. This changes over time. If a whole pile of ones with the mutation are active the numbers of platelets are high. If lots of normal ones are active then the levels drop. However lower numbers doesn't always translate to no clotting issues. Sometimes I have more clotting problems when my numbers are realtively low. There is obviously a lot more involved in clotting than just platelet numbers. The normal megakaryocytes should manage a normal level of platelets so unless you have developed fibrosis of the marrow your levels shouldn't drop too low. It sounds like you have a high proprtion of your normal megakayocytes working for you at the moment. Sounds like a good thing but a check with your heamatologist is always a good idea when things change. Good luck.

Cheers,

SilverET

[stai in reply to SilverET]

Silver ET, thank you for your replay and an interesting comment.

[Nmom in reply to SilverET]

Hi SilverET- can I ask how young you were? My son was just diagnosed Nov 2019 and he is 17. His platelets run 900s-1100s. He’s on low dose aspirin since he is considered low risk. Thank you.

[SilverET in reply to Nmom]

Hi Nmom,

Yep I was about 17 too. It seemed that while I was young this caused me very little issue at all and I wasn't even put on aspirin. Not a lot was known about ET back then. They didn't even know it was due to gene mutations. A lot more is known these days and they know what complications to watch for now too. Get a good specialist and work with them. Sometimes these conditions have been known to burn themselves out instead of getting worse in people who are diagnosed very young so it's not all doom and gloom. I hope things go well for your son. I know my Mum stressed a lot when I was first diagnosed but I was surprisingly Ok with it all so hopefully he can get his head around it too without too much worry.

Cheers,

SilverET

[Nmom in reply to SilverET]

Oh my goodness! Your mom and I could be best friends. My son is totally fine about it and goes on as normal. I am hyper-vigilant about watching him and completely stressed. I hope you are doing well! Thank you for your hopeful response. Blessings to you! Nmom 😊

[hunter5582]

Hopefully the new hematologist will have the expertise in MPNs that you need. The Results of the JAK2 and CALR should be revealing. Would expect the doc also ran MPL mutation check as well. If that polyp had been bleeding - then it could definitely have triggered reactive thrombocytosis. If you already have ET then get reactive thrombocytosis, your platelets are off to the races! Bleeding, inflammation, tissue damage, injury/illness - all can trigger your body to make more platelets. It is a normal response. It become "abnormal" in the context of an existing MPN.

Something to be aware of is that some people with platelet levels as high as your have been experience Acquired von Willebrand Disease. This increases your risk for hemorrhage rather than thrombosis. Current research indicates that it is more common than previously thought and can occur at platelet levels below 1 million. If the doc has not done a von Willebrand panel, which should include ptt/apt - INR (aka prothrombin times), then you might want to inquire.

If it turns out you are JAK2 positive as many of us (me included) are, then you will want to learn more about the role this mutation plays in your body. It goes beyond hematopoiesis.

All the best to you.

[stai in reply to hunter5582]

Thank you Hanter5582 fro your wise comment.

You said "Would expect the doc also ran MPL mutation check as well."

My new Hem is asking for NGS test and on line I find it is Myeloid panel test, maybe it is NGS test that you mentioned?

Bottom line is, this lady Hem knows some things and is willing to act (she is younger person). Previous Hem was into getting the administrative position, when he did, he left to be the director in a big clinic in Portugal. For 13 years he did no tests besides the first one, BMB. A few years ago, I asked about Jak2 testing, he said Jak2 has no clinical value and did not send me.

[hunter5582 in reply to stai]

NGS = Next Generation Sequencing. The Myeloid Panel will be likely looking for related non-driver mutations like ASXL1 and TP53. The three driver mutations are JAK2, CALR, and MPL. It sounds like the new doc is on the ball. I would bet she is checking on all 3 driver mutations, bit it would not hurt to ask.

[stai in reply to hunter5582]

Thank you Hunter5582!