Like to know more. Just told I have Triple Negative MPN.

Hi Lynne,
I am also triple negative ET. You’re the first person that I’ve “met” on this forum who is TN ET. It is rare and thus not well-researched or understood. I’m so delighted to hear from you and about you, and to find you!😀♥️ I’ve had the diagnosis since my BMB in December, 2023.

I’m just seeing this thread. I will read your messages and see what others have said and I’ll add information that I’ve found. I actually attended a webinar about ET today, sponsored by the Leukemia and Lymphoma Society of Canada (I’m Canadian). The only thing that the expert said about TN was that it is still overactivity of the JAK2 pathway, but the cause has not yet been identified.
Thinking of you! I’ll jump back on this thread with links to any that might be helpful.
Take care!❤️

Hi @reneemichelle
So glad to meet you too. I am an Australian and live in Tasmania. How special are we. Just wish for more information. Sharing info is a good thing, especially when not much is known about what we have. Chin up, I have had it for roughly 17 years, just didn't know the exact name was. You have to ask the questions of your specialist, I have only recently found out I am ET TN. Cheers Lynne 🙃 ❤️

I have diagnosis of ET triple negative. So I am missing the common genetic drivers. However, my bone marrow biopsy shows I have a mutation on ASXL1 G646fs. I have read this mutation has been correlated to poor outcomes, ie, progression to MF or AML. Are you aware of any research on this mutation and clinical outcomes or treatments? Thanks!

eloise999, learning that something's "off" is bad enough. Having almost no information about that something makes it a thousand times worse.

Unfortunately this is the reality for ETrs like you and me.

And odds are, your oncologist is probably not an ET triple negative expert.

So all we can do is try to find online information. This can be helpful BUT it's a very imperfect method. One source will contradict another. Some articles are so technical that they're hard to interpret. And there is absolutely nothing that speaks directly to your personal situation.

I hope other triple negative ETrs will chime in, because learning from others' real life experience is so valuable.

We all fear progression. That was my worst fear when I was diagnosed.

I'm much less worried about that now, because from what I've read, that's not a likely outcome for most ETrs.

You are in a scary place, eloise999, but you are not alone, and you will gain strength from others' support and experience. To better days ahead.

Thank you very much for your reassuring words. I really appreciate it.

Hi Lynne,

I see @janemc's wise words about the lack of information. There are a couple of recent articles that provide the latest information about triple negative ET and an annual update on ET in general. They are rather technical, but there are some key messages in them. You can find them with a simple online search:

1. Brief Report "Triple-Negativity Identifies a Subgroup of Patients with Better Overall Survival in Essential Thrombocythemia", Marco Santoro and others "Hematology Reports" 2022, 14, 265-269
from its abstract:
"...clinical features and prognosis (of triple negative ET) have not been described with precision yet...Data from 266 consecutive essential thrombocythemia patients were analysed. Triple-negative patients had a significantly lower symptom load and lower frequency of splenomegaly at diagnosis. The results show that the rate of thrombosis was equal in the two subgroups [triple negative is one subgroup, other subgroup is folks with one of the 3 driver-gene mutations]. Overall survival was slightly better in the triple-negative group of patients. "

2. "Triple-Negative Essential Thrombocythemia: Clinical-Pathological and Molecular Features. A Single-Center Cohort Study", by Daniele Cattaneo and others, Frontiers in Oncology, 2021 Mar 12; 11:637116.
Very technical - looks at the bone marrow morphology and the clinical-laboratory parameters of triple-negative ET patients and the molecular profile to idetnify potential clonal biomarkers.

3. "Essential Thrombocythemia: 2024 update on diagnosis, risk stratification, and management", by Ayalew Tefferi and others, American Journal of Hematology, 2024, volume 99, Issue 4, p.697-718.
This annual update article gives an overview for all ET (JAK2, CALR and MPN as well as TN). The article cites "Mayo-Florence" studies of 2000 patients. In the concluding remarks, the authors state that those studies "were concordant in their identification of triple-negative driver mutational status as a particularly favorable risk group, in terms of both thrombosis and disease transformation [to AML or MF]...Accordingly, it is reasonable to recommend a highly conservative treatment approach in triple-negative ET that is devoid of cytoreductive drugs...The outstanding prognosis in triple-negative ET makes it difficult to subject such patients to clinical trials with drugs devoid of demonstrable disease-modifying activity."

Anyways, of course since "very few papers are presently avalable on triple-negative ET, which is basically described as an indolent [slowly progressing] disease" (stated in the abstract of the second article), one has to be cautious. It reflects the information currently available, and the statements are relatively favourable re triple-negative ET. I appreciate getting as much information as possible, to ask my O/H the questions that are important to me.

It's good to keep reading, asking questions and sharing so that we can be our own best advocates!

Take care!!👍😀❤️

Thank you for the information, it certainly helps. One day at a time. Cheers Lynne :-]

reneemichelle, thank you so much for these citations.

For the third item, this link will take you to the full article:
https://doi.org/10.1002/ajh.27216
An overview of research from all over the world, this provides the latest findings on multiple aspects of ET. Relevant to all of us, not just those who are triple-negative.

The article touches on questions we discuss here all the time. Will ET shorten my life? What are my risk factors for clots, or for progression to leukemia? For whom is low-dose aspirin or HU helpful? What should I do if I need to prepare for surgery?

I offer the following quote for your consideration:

There are, to date, no controlled studies that implicate either HU or busulfan as being leukemogenic in either ET or PV. Similarly, the two largest non-controlled studies in ET and PV do not support the concern that leukemia might arise from the use of HU and there is additional evidence to that effect from long-term studies of patients receiving HU for sickle cell disease.

Thanks again, reneemichelle.

@reneemichelle

I just learned I am triple negative likely ET. I am scheduled for a bone marrow biopsy next week for confirmation. Or it could be mylofibrosis. My platelets are 965 and I have anaphylaxis response to aspirin so I am anxious about how blood thinning will be treated. I’m not sure why but my Dr suggested I might need a bone marrow transplant. That freaked me out a bit. I am 63 female.

@mmkennedy

mm -- you are in a really tough spot right now. Of course you feel baffled and scared.

Please know that many oncologists use bone marrow biopsies to confirm diagnosis. That one's been ordered for you doesn't have sinister connotations.

The day my ET was diagnosed, I too was warned a bone marrow transplant might be needed.

Maybe oncologists say this so that we will take our diagnosis seriously, and understand the importance of doing whatever we can to support the health of our bone marrow?

I get the scare tactic, but a message of hope would also be welcome!

I'll bet there will be a good alternative to taking aspirin.

Another med that may be mentioned to you is hydroxyurea (HU), which helps our bone marrow by tamping down the manic overproduction of platelets that is ET.

HU has greatly restored the quality of my life.

You can enter TPN in the search box at the top of this page to see other discussions on TPN ET.

You can learn a lot about HU in the same way.

You are NOT alone, mm.