Essential thrombocythemia

ET is definitely classified as a blood cancer and I have read MPN experts refer to it as a “chronic leukemia” as opposed to an “acute leukemia. “

I realize I’m replying to myself but I hope everyone can see this. I’m starting to have problems with neuropathy of my feet so the doctor put me on Gabapentin which is for neuropathy pain. The problem for me is sleeping. When I’m in bed and my legs touch it sets my nerves off off and I experience cramps and tingling. Sometimes the tingling is so bad I can “hear” my nerves complaining. Like white noise in my brain. Just annoying. When it starts I have to sleep on a recliner. Within the last month I’ve started having back pain. I have MDS/MPN-RS-T and one of the diseases in that overlap disease is myelofibrosis. I’m heading off to an Orthopaedic doctor for X-rays to determine if my back pain is coming from “weakened bones” or is muscular in nature. It never stops does it?

@mjpm2406, you might benefit from joining the discussions in the Neuropathy group https://connect.mayoclinic.org/group/neuropathy/

In particular this discussion:
- Neuropathy Pain at Night: What helps? https://connect.mayoclinic.org/discussion/night-pain-2/

It’s my understanding that bone marrow transplants are very risky as many patients reject the marrow. Not everyone is eligible. I believe age is a factor in eligibility but not sure. I have ET and am holding off taking hydrea as long as possible due to its potential side effects. Hoping to take Pegasys which is a pegalated interferon when the time comes. Some doctors call it a chronic leukemia because it is a blood cancer. Some doctors still say it as a “blood disorder” even though it’s been classified as a cancer since 2008 when the mutations were discovered.

Hi Tessa,
I'm glad you reached out, seeking others' experience with ET - a rare and not well known blood cancer. Like you, I'm mostly asymptomatic although I do struggle with fatigue. As I understand it, most people find out that they have ET from having a CBC, unless they unfortunately experience an "episode,"as in a blood clot or stroke. For many years, I have had ET and because of the level of my platelets, I'm taking a daily 81mg aspirin.--very good news. There are many things to consider is starting Hydroxyurea, In my experience, Drs do not encourage its use until it's essential, You mentioned wanting to have children and I'd like to recommend that you read up on ET, so you'll get acquainted with the side effects. While you didn't mention it, 50% of people who have ET have no gene mutations; I'm one. They just don't know enough about it. Read up and continue to ask questions. Stay positive! Best always,

I am a 68 yr old, originally diagnosed with ET in late 2017. After a Bone Marrow biopsy, turns out I have the next level up...PMF.
Taking Hydroxy for 2 years now. Side effects they can not explain...Purple Toes...seems to occur more in Cold weather....WHAT....I live in Phoenix! Cold is 60 degrees...lol...tried Cryoglobulin tests...negative....standard answer I get is....Might be Hydrea...or just your cancer. In other words they do not know. Get used to that answer! I have been to two top 10 in the world specialists (well for this range of blood cancers because we are a small group) in PHX...at Mayo Clinic and MD Anderson. Neither ever saw Purple Toes before and one of them found only 1 other case in the literature.....never figured out what it was. Doc wanted to try switching to Jacafy...because I do have the Jack2 mutation...I found out it would cost me $7000 a month....SO I thanked her for her Professional GUESS, but insisted on staying on Hydrea. Remember two things 1) Doctors are leading YOUR Team....this is your cancer...OWN it. You get to make the ultimate decision. 2) these oral chemistry are treating Symptoms you may be having....if they were a cure, heck I would find a way to pay $7000 a month. Most recently I have developed lung congestion...confirmed thru a CT SCAN and I am off to a Pulmanary specialist to get his best guess. I will tell you that WHOEVER is treating you probably specializes in some other more prevalent cancer and Really is not up on the latest literature...I forward copies of MPN newsletters to mine. And whomever they refer you to...well...chances are they have never seen your cancer either. So expect a lot of head scratching. In summary, here is what I have learned in two years
- WE have an extremely rare disorder. NOONE specializes in your specific cancer.
- this is MY cancer and I use doctors opinions to form my own decisions. I may not be an expert, but neither are they.
- listen and learn...read and reach out with blogs and groups like this....it really does help. And MOST Importantly...stay positive! You folks with ET are lucky....when mine got stepped up to PMF...I discover a bone marrow transplant was ultimately my only cure!
Then I read internet searches on that and it scared me to death! Turns out Internet is full of old information, which is why you want current literature and conversations with people who have similar type cancers. Finally...GOOD LUCK! I sincerely hope this helps just one person.

Talked to some Specialists on Bone Marrow transplants and the level or risk depends on the stage of your cancer, your age and general health. Risks of marrow acceptance are dependent upon donor match, General stage of cancer and your overall health. Those who wait until they NEED a transplant are the highest risk. I have heard everything from Leukemia to Disorder...just shows you the level knowledge that is out in the general physician community.

Hi, yes...I take Hydrea and the instructions are so tough...DO NOT even let my female family or friends of child bearing age touch it! My 30 yr old niece was diagnosed with ET at 15...and takes a Chemo drug to manage symtoms. She wants to have children and would have to stop the drug for a while before getting pregnant up to birth. So that automatically puts her in high risk and bring symtoms back. Tough choices. If you can get to an MD Anderson or Mayo Clinic....you have a better chance of finding a doctor who specializes in blood disorders...find one....good luck

MPN newsletter gives you the latest on research and clinical trials....great information.

In response to earlier posts I was asked to post bone marrow biopsy results, hope this is not too long. Diagnosed ET 11/19, platelets exceeded one million briefly, daily hydroxyurea knocked that down to 760, has slowly risen to 900. My Hematologist: this is basic ET, won't be the cause of my demise and probably won't transition to other forms. Other than minor fatigue I feel fine. In the last six months I have switched to a plant based diet, increased daily exercise, and struggled to lose 20 lbs. For my efforts my blood pressure suddenly increased resulting in doubling my ace inhibitor and a .325 aspirin nightly. Not quite the results I was looking for! Word of advice: if you're getting a biopsy get anesthesia! A friend did it with just numbing and had a week of pain. The procedure is on utube.

BONE MARROW CORE BIOPSY, CLOT SECTION AND ASPIRATE SMEARS:
--- HYPERCELLULAR BONE MARROW WITH MEGAKARYOCYTIC HYPERPLASIA,
CONSISTENT WITH TREATED ESSENTIAL THROMBOCYTHEMIA
--- BLASTS: NOT INCREASED.
--- NO OVERT EVIDENCE OF FIBROSIS

PERIPHERAL BLOOD:
--- THROMBOCYTOSIS

Comments:
Mild megaloblastic changes seen on myeloid precursors and neutrophils,
likely due to the drug effect of hydroxyurea. Cytogenetic and molecular
studies are pending.

CLINICAL HISTORY:
This is a 72y.o. male with essential thrombocytosis (diagnosed in
10/2019) with CALR mutation on hydroxyurea and aspirin.

INTERPRETATION:
BONE MARROW CORE BIOPSY/ ASPIRATE CLOT SECTION:
Adequacy: Adequate for evaluation.
C
ellularity: 40-50%
Erythropoiesis: Adequate
Granulopoiesis: Mildly increased.
Thrombopoiesis: Megakaryocytes are increased and many of them are
mostly large with clustering focally.
Trabeculae: Normal
Other Findings: There is no overt evidence of fibrosis.

BONE MARROW ASPIRATION (GIEMSA STAIN):
Adequacy: Adequate for evaluation.
Blasts: 2%
M:E ratio: 3.2:1
Erythropoiesis: There is no dysplasia.
Granulopoiesis: Mildly increased with some megaloblastic changes.
Thrombopoiesis: Megakaryocytes are increased, many of them are large
in size.
Plasma Cells: Not increased.
Lymphocytes: Small and mature.

IRON STAINS ON CLOT SECTION AND ASPIRATE SMEAR:
Storage iron: Adequate
Sideroblastic iron: Present
Ringed sideroblasts: Absent

PERIPHERAL BLOOD (GIEMSA STAIN):
White Blood Cells: Neutrophils show occasional hypersegmentation.
Lymphocytes are morphologically unremarkable.
Red Blood Cells: Slightly decreased and slightly macrocytic with
minimal aniso
cytosis. There is no overt polychromasia. No significant
schistocytes or spherocytes identified.
Platelets: Markedly increased.