1. < Blood Cancers & Disorders

Essential Thrombocythemia: Looking for information and support

Posted by shenriq @shenriq, Jun 4, 2018

I was recently diagnosed with Essential Thrombocythemia, a rare incurable blood cancer. Platelet count aside, I am asymptotic. This current condition morphed from (constitutional) thrombcytosis, something I’ve lived with for 25+ years. While the new diagnosis was the result of a bone marrow aspiration and biopsy, my age was an additional factor, which was completely disarming, having been walking around unwittingly for the past 8 years! While at the low end of risk for clots, heart-attacks and stroke, nothing has truly changed - except the “C” word. No chemo yet, but active discussion about hydroxyurea. Uncertainty about ET is anxiety provoking and swoethatl, but I’m feeling betrayed by my blood. I’m looking for all information about ET, the chemo and support.
Thanks!

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mohandas | @mohandas | May 23, 2023

I am on Hydroxy urea since early 2019. Platelets count fluctuates. So does the dosage of Hydroxy urea. 500 mg daily and additional 500 mg on alternate days. Aspirin 75 mg and antacid to keep gastro intestinal track in check is regular medicines. Physical activities throughout the day is must. A minimum of 10000 steps a day keep the blood flow normal. Plenty of water, about 4 liters helps in haemo-digestion of extra platelets I guess. Staying away from toxicity is most important as we can't afford to get sick with other chronic illness.

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1 reply
remo1110 | @remo1110 | May 23, 2023

I was diagnosed. with ET 3 years ago. I have been taking hydrea since I first saw the oncologist. the condition is not curable but can be kept under control with the medication...
Hydrea is very easy to tolerate not a lot of problems sometimes lack of apatite and a bit of tiredness. Hope this helps a little.
good luck

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mjpm2406 | @mjpm2406 | May 24, 2023
In reply to @bonnieshaffer "I also have MDS/MPN with RS and T. My platelets shot up to 1,100 in March...." + (show)
@bonnieshaffer

I also have MDS/MPN with RS and T.

My platelets shot up to 1,100 in March. Started taking HU 1000 mg a day 7 days a week.

By May my platelets were down to 300. I'm now taking 500mg every other day.

I haven't felt any side effects and am glad the HU worked so well as it is the treatment with the least number of side effects.

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I was misdiagnosed at the VA Hospital on Long Island with ET in early 2018. I asked the Hematologist when she would perform a bone marrow procedure. She said, “no need for a bone marrow procedure because I know exactly what’s wrong with you and I know exactly how to treat ET.” Her problem was I’m a MIT Researcher and I know you cannot diagnose any type of blood cancer without a bone marrow procedure and a molecular panel or genetics test. So I contacted Sloan and had the bone marrow procedure, molecular panel and a genetics test done. My final diagnosis in 1998 was MDS/MPN-RS-T. Sloan has seven cases of MDS/MPN/RS-T. Most hospitals have no cases because this sucker is extremely rare. It appears my blood disorder is mutating because my immature white blood count is running at 9.5% of my white cell count. So not looking forward to a stem cell transplant. Hopefully, that will not happen. I have the JAK2 mutation which is common in MDS and MPN disorders. I also have the SFB31 and SRSF2 splicing gene mutations. I understand it’s rare to have both the SFB31 and SRSF2 mutations.

My platelet count has been normal for the last 4 years with my current dosage of Hydrea. My Oncologist at Sloan thinks I’ll need another bone marrow procedure in the near future because my immature white cell count is elevated but he doesn’t appear to be overly concerned YET. And I’m not worrying about it until he says I have something to worry about.

Hope your blood disorder stabilizes.

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1 reply
bonnieshaffer | @bonnieshaffer | May 24, 2023
In reply to @mjpm2406 "I was misdiagnosed at the VA Hospital on Long Island with ET in early 2018. I..." + (show)
@mjpm2406

I was misdiagnosed at the VA Hospital on Long Island with ET in early 2018. I asked the Hematologist when she would perform a bone marrow procedure. She said, “no need for a bone marrow procedure because I know exactly what’s wrong with you and I know exactly how to treat ET.” Her problem was I’m a MIT Researcher and I know you cannot diagnose any type of blood cancer without a bone marrow procedure and a molecular panel or genetics test. So I contacted Sloan and had the bone marrow procedure, molecular panel and a genetics test done. My final diagnosis in 1998 was MDS/MPN-RS-T. Sloan has seven cases of MDS/MPN/RS-T. Most hospitals have no cases because this sucker is extremely rare. It appears my blood disorder is mutating because my immature white blood count is running at 9.5% of my white cell count. So not looking forward to a stem cell transplant. Hopefully, that will not happen. I have the JAK2 mutation which is common in MDS and MPN disorders. I also have the SFB31 and SRSF2 splicing gene mutations. I understand it’s rare to have both the SFB31 and SRSF2 mutations.

My platelet count has been normal for the last 4 years with my current dosage of Hydrea. My Oncologist at Sloan thinks I’ll need another bone marrow procedure in the near future because my immature white cell count is elevated but he doesn’t appear to be overly concerned YET. And I’m not worrying about it until he says I have something to worry about.

Hope your blood disorder stabilizes.

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Hi there,

Yes, this diagnosis does seem to be extremely rare! I don't qualify for RARS-T because I don't have 20% ringed sideroblasts (I thought the cut off was 15%) according to my Oncologist.

My Oncologist basically said I will need a bone marrow transplant in 5 years due to my age (61).

I told him I don't think I want to go through that and he said we could revisit later on.

He couldn't really give me a prognosis due to the fact that there is not really a cohort of people large enough to study.

My mutations are: SF3B1, DNMT3A, TET2, CUX1 and JAK2.

I'm certainly glad to meet you and know you are there!

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1 reply
crhilston | @crhilston | May 25, 2023
In reply to @loribmt "Good morning, @crhilston. In another reply you wrote that you’ve recently found out you have acute..." + (show)
@loribmt

Good morning, @crhilston. In another reply you wrote that you’ve recently found out you have acute myeloid leukemia (AML). I also had this diagnosis 4 years ago. I’ll be honest with you, this one is a bugger.
I think this informational article from our Mayo website will help explain what’s happening with your blood better than I’m able to do… Here’s an excerpt:
“Your bone marrow produces blood cells. Acute myelogenous leukemia occurs when a bone marrow cell develops changes (mutations) in its genetic material or DNA. A cell's DNA contains the instructions that tell a cell what to do. Normally, the DNA tells the cell to grow at a set rate and to die at a set time. In acute myelogenous leukemia, the mutations tell the bone marrow cell to continue growing and dividing.

When this happens, blood cell production becomes out of control. The bone marrow produces immature cells that develop into leukemic white blood cells called myeloblasts. These abnormal cells are unable to function properly, and they can build up and crowd out healthy cells.”
https://www.mayoclinic.org/diseases-conditions/acute-myelogenous-leukemia/symptoms-causes/syc-20369109
While no one can predict your life expectancy with this diagnosis, what I can tell you is that once the abundance of defective, immature cells start outnumbering the healthy cells, if not treated, this condition can develop rapidly.
Taking into account your age, your hematologist may feel that some of the more aggressive treatments aren’t appropriate. But you may want to ask about potential chemo treatments or abrogating drugs that can slow the progression of the proliferating cells.

Illnesses like this can come with a lot of questions so please, don’t hesitate to fire away if anything comes to mind that you’d like to know. I’m here for you anytime.
What has your hematologist discussed with you?

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I had an appointment with an oncologist, MD, to evaluate the results of my bone marrow biopsy. The diagnosis is final - acute myeloid leukemia. My family and I will discuss what I want to do next. A cure is not an option, however, there about three treatment plans to consider; she explained these in detail including the many possible side effects. So, any comments will be most welcome. I will make my decision at my next appointment on June 8. Thanks!

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3 replies
nohrt4me | @nohrt4me | May 26, 2023
In reply to @mohandas "I am on Hydroxy urea since early 2019. Platelets count fluctuates. So does the dosage of..." + (show)
@mohandas

I am on Hydroxy urea since early 2019. Platelets count fluctuates. So does the dosage of Hydroxy urea. 500 mg daily and additional 500 mg on alternate days. Aspirin 75 mg and antacid to keep gastro intestinal track in check is regular medicines. Physical activities throughout the day is must. A minimum of 10000 steps a day keep the blood flow normal. Plenty of water, about 4 liters helps in haemo-digestion of extra platelets I guess. Staying away from toxicity is most important as we can't afford to get sick with other chronic illness.

Jump to this post

I totally agree that exercise and water intake are important to feeling well and having more energy. For an elderly person like me, the doc said 7,000 steps was a good goal. I use an elliptical machine because I have some back problems. I also find 30 minutes of daily yoga very helpful for mobility and stress. And reducing sugar and starches made me less sleepy. We can't cure ET, but I wish doctors would underscore how much we can do to improve quality of life.

REPLY
nohrt4me | @nohrt4me | May 26, 2023
In reply to @crhilston "I had an appointment with an oncologist, MD, to evaluate the results of my bone marrow..." + (show)
@crhilston

I had an appointment with an oncologist, MD, to evaluate the results of my bone marrow biopsy. The diagnosis is final - acute myeloid leukemia. My family and I will discuss what I want to do next. A cure is not an option, however, there about three treatment plans to consider; she explained these in detail including the many possible side effects. So, any comments will be most welcome. I will make my decision at my next appointment on June 8. Thanks!

Jump to this post

I am so glad you got good explanation of your treatment options.

Something I would consider at my age (70): What treatment is going to give me the most time to live my life and be the least drain on time and headspace?

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Rhonda Gregory | @rjgregory440 | May 26, 2023
In reply to @crhilston "I had an appointment with an oncologist, MD, to evaluate the results of my bone marrow..." + (show)
@crhilston

I had an appointment with an oncologist, MD, to evaluate the results of my bone marrow biopsy. The diagnosis is final - acute myeloid leukemia. My family and I will discuss what I want to do next. A cure is not an option, however, there about three treatment plans to consider; she explained these in detail including the many possible side effects. So, any comments will be most welcome. I will make my decision at my next appointment on June 8. Thanks!

Jump to this post

We're all here for you!

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mjpm2406 | @mjpm2406 | Jun 1, 2023
In reply to @bonnieshaffer "Hi there, Yes, this diagnosis does seem to be extremely rare! I don't qualify for RARS-T..." + (show)
@bonnieshaffer

Hi there,

Yes, this diagnosis does seem to be extremely rare! I don't qualify for RARS-T because I don't have 20% ringed sideroblasts (I thought the cut off was 15%) according to my Oncologist.

My Oncologist basically said I will need a bone marrow transplant in 5 years due to my age (61).

I told him I don't think I want to go through that and he said we could revisit later on.

He couldn't really give me a prognosis due to the fact that there is not really a cohort of people large enough to study.

My mutations are: SF3B1, DNMT3A, TET2, CUX1 and JAK2.

I'm certainly glad to meet you and know you are there!

Jump to this post

I told my Oncologist that I would not go through a stem cell transplant after 80. I’m almost 77. He said we’ll discuss this when and if your MDS/MPN-RS-T mutates or no longer responds to the normal meds to treat this disorder. Sloan has the RS number at 15%. So, do you experience fatigue during the day? I need a 30 minute nap every day. I also have Chronic Sensorimotor Axonal Polyneuropathy, CMT2 and CMT4B. The Sensorimotor Axonal Polyneuropathy is an acquired disease while the CMT disorders are hereditary. My mothers family has a six generation history of this disorder. CMT comes down on the X chromosome. No one ever had a genetics test done in the past because whole Exome Sequencing wasn’t around. I went with my three daughters to Weill Cornell in Manhattan to undergo genetic testing. Results indicated one daughter had no mutations. Daughter 2 had the CMT2 mutation. Daughter 3 has both the CMT2and CMT4B mutations. So this sucker that has plagued my mother’s family continues to plague my family.
Glad to finally hook up with someone with a similar case if MDS/MPN. Our mutations aren’t the same but I assume our symptoms may be close.

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1 reply
Mentor
Lori, Volunteer Mentor | @loribmt | Jun 1, 2023
In reply to @crhilston "I had an appointment with an oncologist, MD, to evaluate the results of my bone marrow..." + (show)
@crhilston

I had an appointment with an oncologist, MD, to evaluate the results of my bone marrow biopsy. The diagnosis is final - acute myeloid leukemia. My family and I will discuss what I want to do next. A cure is not an option, however, there about three treatment plans to consider; she explained these in detail including the many possible side effects. So, any comments will be most welcome. I will make my decision at my next appointment on June 8. Thanks!

Jump to this post

Hi @crhilston I just wanted to pop in this morning to see how you’re feeling. With newly diagnosed AML, in the early stages, you’ll most likely be feeling some fatigue and maybe shortness of breath. Do you have any other symptoms?

I’d be very interested in learning what your doctor will recommend for potential treatments for you. I know there are new abrogating meds on the market. Would you mind sharing after your appointment?

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