Essential Thrombocythemia: Looking for information and support

I'm also CALR. Started on 500 mg HU daily when platelets approached 800. Took a year and an increase to 1000 mg M-W-F to get them to 400s. That's where I've been for the last five years (I'm 69 now) with one or two jumps to the low 500s. Very mild side effects with HU that exercise and diet changes helped.

Doc said that CALRs are less prone to clotting, so she's happy with mu maintenance dose. Wondering what your hemo's platelet target for you is.

Also, seems to me that CALR patients take longer to respond to meds than those with JAK2. Anyone else notice this?

I'm so glad HU is working for you and you're not having side effects.

I was diagnosed in November '22 with the CALR mutation and have been on 1500mg/daily of Hyrdroxyurea for a while to bring my platelets down from 1700 into normal range with an elevated white blood cell count the whole time, but then when it was eased up to 1000mg the white blood cell count went back to normal, but the platelet level worked back up into the 500's, so now I've been on 1000mg 4 days per week and 1500mg 3 days per week to see if that gets it back down without raising the white blood cell count back up. Going every 6 weeks for a blood draw now. No side effects the entire time, except for some bloating for the first couple weeks from the Hydroxyurea. I'm very active and have not lost any energy or had weight gain.

Yes, ultrasound, needle biopsy and follow up ultrasound a year later + labs that showed normal thyroid function. They will ultrasound again in a few years unless changes. I think thyroid function tests will be ongoing. I told my primary I'd rather not have another specialist involved unless it was necessary, so I nixed an endocrinologist for now. These people are so silo'd into their specialties that it becomes a full time job trying to coordinate communication. Already got a primary, hematologist, cardiologist, and dermatologist.

Hi Jewelfaux, I think the answer about "does it make a difference to have diagnosis done via blood test or BMB" is "it depends". When I first saw my MPN specialist roughly a decade ago after high platelets and Jak2 mutation, I was told that based on blood work, ET was the probable diagnosis. My doctor advised that sometimes other MPNs (PV, MF) can present similar to ET in the bloodwork and the only way to truly diagnosis ET is via a BMB. Having said that, he also advised at that point, regardless of diagnosis, his treatment plan for me would be the same at that time (which was aspirin only).

I opted not to have a BMB at that time. As the years passed, I continued to ask about BMB, with generally the same response from my specialist. I did express concerns about potential progression, and he was clear that the BMB would be the best way to better understand this, as what can be uncovered in the blood work is much more limited than a BMB. I ultimately opted for the BMB, which showed that I had signs of both ET and pre-fibrotic MF, which to me was very important to know. Treatment has come a long way in the last decade, and as I understand it, interferons can be disease-modifying. So with the potential of MF on my horizon, I have been contemplating this course.

So the "it depends" answer is really: what is important for you to know about your ET/MPN? It is not "required" per se, but can be extremely valuable. I don't regret waiting to have one, but I am also glad I ultimately did. Knowledge is power.

Hugs to you my friend.

Laura

Did your family doctor do the needle biopsy?

I also had a benign goiter and non malignant nodules. I see an excellent endocrinologist. I do have thyroid labs done also, Each time I go he personally does an ultrasound and compares it to the previous ultrasound. Last time he said to come back in two years. When I see him next I will bring up the subject of ET JAK2 and Hydroxyurea.
Eileen

Two things on dosing:

1. Some doctors, especially in the past, have given big amounts of HU because platelets are very high. This happens less often because patients do better if they start on lower doses.

2. Patients do not respond the same way to HU. It takes bigger doses to get some patients into acceptable levels. More research is needed here that might offer better dosing guidelines, but the time, energy, and $$$ seems to go toward new drug research, even though many patients can't afford new drugs.

2 bone marrow tests

I guess it depends on the hematologist/oncologist’s experience. I was never offered the option - I requested it. It didn’t make a difference to treatment, but it did rule out advancing myelofibrosis.

I am amazed at the amount of HU some people are taking. I take 500 mg daily…. My platelets peaked at 800,000. Once on HU they have dropped to 275,000 and are stable. I am also JAK2 positive.