Recently diagnosed with ET

[blueseas45]

Been reading on this site for awhile trying to learn more about this. I just had a six month blood test and all this came up. I appreciate everyone telling their story. My Recent lab results. I am still in still in shock about this. This is what my oncologist came up with on blood tests. He still hasn't done a bone marrow test, so I am not sure how he knows it's ET for sure.. I am supposed to return July 9, 2021 for blood tests again to se if my platelet count has dropped. I have a visit to the urologist August 18, 2021. My father passed away with prostate cancer.

Recent Labs

Component Name 05/28/21 1232 05/19/21 1104 11/18/20 1002

WBC 13.3* 13.3* 11.3*

HGB 12.6 13.5 13.8

HCT 40.6 44.1 45.8

PLTCOUNT 767* 860* 509*

Assessments and Recommendations:

1. Essential Thrombocytosis

2. Leucocytosis

3. Neutrophilia

4. Elevated PSA

-leukocytosis with neutrophilia and thrombocytosis, workup done showed him to be positive for CALR mutation, he tested negative for JAK2 V617F mutation, Exon 12 mutation and MPL mutation. BCR-ABL PCR was negative.

Iron studies showed good iron stores. ESR and CRP were within normal limits.

He had a CT scan lung cancer screening which did not show any lung nodules.

This is consistent with a diagnosis of CALR positive essential thrombocytosis, he has elevated platelet count of 773, I have discussed with him that patients with myeloproliferative neoplasms with elevated platelet count there is a risk of thrombosis, patients with CALR mutant thrombocytosis have a relatively low risk of thrombosis compared to the other in today's however I will recommend lowering the platelet count with hydroxyurea.

I discussed the side effects of the drug with the patient in detail including but not limited to and depression of blood counts, ulcers, nausea, vomiting, diarrhea, he expresses understanding and agrees to proceed.

A printout of the drug was provided to the patient.

I will have him return to clinic in 3 weeks with CBC and CMP.He he has been noted to have a rising PSA, I have advised him to see his urologist for a biopsy.

[blueseas45]

From those test results is it for sure that I have ET?

[cmc_ufl]

Hi there. Since your notes have indicated that you tested positive for the CALR mutation, it appears you likely do have ET. It also appears your physician indicated in the notes that your presentation is consistent with CALR-positive ET. ET is a very manageable condition, and youve in the right place looking for information.

I am not a physician, but here is some helpful information regarding the various MPNs.

Your BCR-ABL test was negative, which ruled out chronic myelogenous leukemia.

The fact that you were JAK2-negative and did not show elevated red blood cell counts ruled out PV (PV almost exclusively presents with a JaK2 mutation).

The fact that you’re not anemic makes PMF highly unlikely.

ET typically presents with only elevated platelets, but under certain conditions, elevated WBC can also occur. It is possible that you have ET also presenting with elevated WBC, or that you have ET along with something else causing the elevated WBC. I’m sure your physicians will investigate this further to determine exactly what is going on.

Definitely get the biopsy recommended due to the elevated PSA, especially given your family history of prostate cancer.

This forum is an excellent replace to get support and reliable information. Let us know how you get on.

[blueseas45 in reply to cmc_ufl]

Thank you very much for the information. I am a little overwhelmed with this. I have any appointment next month for the urologist. My psa jumped from 4.5 to 9.8 from last November to may of this year. I love this site and all the friendly people and advice from all of you that been dealing with these horrible blood related diseases. God bless all of you.

[blueseas45]

I have so many questions. I live in the country and have a lot of grass to cut. I see where they say stay out of the sun with hydroxyurea! I have at least 3 to 4 hour of cutting grass in the sun. Can I still do that with high spf sunscreen. This skin cancer scares me. Also does this mean I can never go fishing anymore? I feel like this has stolen my life.

[Mazcd Partner MPNVoice in reply to blueseas45]

Hi Blueseas45, welcome to our forum. I would suggest that you read as much as you can on our website mpnvoice.org.uk, there is lots of useful information which I am sure will help you. This page about ET and how it is diagnosed will help mpnvoice.org.uk/about-mpns/...

With regards to going out in the sun, you do need to take care in the sun your skin may be more sensitive to the sun whilst you are taking hydroxycarbamide so it would be wise to avoid exposure to the sun, using sunscreen and wearing protective clothing and a hat.

best wishes, Maz

[blueseas45 in reply to Mazcd]

Thank you Mazcd. I guess I can wear a long sleeve shirt, cowboy style hat and tons of sunscreen. I usually where gloves while cutting grass with my tractor or my mowers. I was looking for ward to going to Florida fishing this year with my son. Thank you for the link I will read it all. If my platelet count would drop down to lets say in the 200's and I am only on aspirin could I get in the sun?

[Mazcd Partner MPNVoice in reply to blueseas45]

Best to discuss that with your haematologist. Maz

[blueseas45 in reply to Mazcd]

Ok. Thank you

[Janis12 in reply to blueseas45]

Hi, like you I am an outdoor person diagnosed with ET just over a year ago. I worried about the sun issue as I cycle three days a week for about 5 hours each day. I am also a keen walker and I have a large garden. As I live on my own there is no choice, I must go out regardless of the weather. I cover up where I can but to be fair doing a 50 mile bike ride on a warm day definitely needs to be in shorts. So I managed to get sun block on prescription from my GP and I use it all year round and I pray that this is going to be sufficient protection. At my next haematology appointment I am going to ask if I could have an annual dermatology review (shy kids get nowt) as I do not trust myself to spot 'unusual lesions'. My quality of life is very important to me and being outdoors at any time of year is therapeutic. Good luck to you and get that sun screen slapped on and off you go!!

[blueseas45 in reply to Janis12]

Hi Janis12. So good to hear someone is doing outdoor activities. I live by myself also, these things have to be done. Is that sun block a real high spf? You make me feel a lot better. Sitting in the house all the time is not my thing. I will talk to the cancer doctor friday about the blocker. Impressed with your cycling! Hope all keeps going well for you. Hugs

[Janis12 in reply to blueseas45]

Hi, I use factor 50 even in the winter, cycling is pretty harsh on the skin particularly in the north of England where I live. The sun cream that is available on prescription needs re applying every 2-4 hours depending on which one they give you (also factor 50) but when I am out cycling I use one I buy myself called Reinmann P20 and I get the factor 50 version of that. One application lasts 10 hours which is more convenient for an outdoor activity. Once I get a referral to dermatology I will 'pick their brains' just to make sure I am doing the best I can to continue with my outdoor life style. If it did become a problem then I would be looking at asking my consultant to take me off Hydroxycarbamide and treating me with something else that does not have the same side affects. Fingers crossed, all the best.

[blueseas45 in reply to Janis12]

Thanks Janis12. Application every 10 hours sounds great. I will look into it. Happy cycling

[hunter5582]

Hello and welcome to the forum Glad you found your way here.

As others have indicated, ET is a manageable condition. For those under age 60 in the absence of other risk factors the recommendation would usually be aspirin-only. In the over 60 (or in some protocols 65) or other higher risk groups, there are two first-line choices for cytoreduction, hydroxyurea and PEGylated interferon. Your doctor would likely review both options with you if you ask about your choices.

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

legeforeningen.no/contentas...

The target for cytoreduction varies between hematologists. Some use a "normal" figure of 450 or 400. Others actually use 600. You have to weigh the benefits of cytoreduction to the intrinsic risks of cytoreductive medications.

silvermpncenter.weill.corne...

Treatment plans are best when they are individualized. We each present differently with our MPNs and cooccurring conditions. We each react differently to each of the treatment options. You will hear from some that they tolerate hydroxyurea well and benefit from it. Others, myself included, cannot tolerate it. I experienced toxicity even at low doses. I appear tp be tolerating the PEGylated Interferon far better. Others cannot tolerate PEG-IFN at all.

Since you are considering HU, here are a couple of links to reliable sources of information on it. These same sites can be used to look at info on PEG-IFN.

drugs.com/monograph/hydroxy...

online.epocrates.com/drugs/...

These same sites can be used to look up info on PEG-IFN if you are interested. The thing to know is that each of the options for treating ET has a risk/benefit profile. You have to evaluate each based on your treatment goals and priorities. Ultimately it is your preferences that need to drive the treatment decisions.

The good news is that you have time to sort this all out as you learn more about ET. I was diagnosed with ET about 30 years ago. It progressed to PV about 8 years ago. Now in my mid-60s I am doing fine, albeit with a few challenges and interesting health-learning opportunities.

All the best to you on your MPN journey.

[blueseas45 in reply to hunter5582]

Thank you hunter5582 for all the great links ( I will be reading them today). I am 73 years old, so I guess my age doesn't help much. My doctor really never went over any other drug options (think he's just trying to get the platelet count down right now). I love your posts they are so informative all the time. I am so glad you are keeping ET under control. The sun issue is a real problem for me (I love the sun).

[kp27kathleen]

My numbers match yours almost exactly. I have been on HU for 5 yrs and have not had any problems. I live in sunny Arizona and have very fair Irish skin, I have had a few skin cancers on my face but not the serious type. I go to the dermatologist twice a year so he keeps an eye on anything suspicious. Now my numbers hang around 500 and the doctor is happy and so am I. My HU is 500 mg once a day. Enjoy your life!!

[blueseas45 in reply to kp27kathleen]

Hi kp27kathleen. Do you do any outdoor activities? Most the things I like to do is outside. Your taking the same dose I am right now on HU. I hope your numbers keep improving.

[blueseas45]

I went to the oncologist yesterday 07/09/2021 and my platelet count dropped 111 points to 657 from 767. I was a little disappointed it did not drop more, but at least it did not go up. Have a blood test again in two weeks and a blood test and oncologist appointment in four weeks.