I was diagnosed with essential thrombosis in 2018. I had the biopsy that confirmed JAK2. My platelet count has always been somewhat normal as high as 450 but right now it’s only 300. However, I have many of the secondary symptoms, such as erythromyalgia, although I have not been diagnosed 100% with Erythro myalgia, I have the symptoms, swollen hands, and feet tingling., Severe burning and itching and lesions for arms, fatigue, etc. what concerns me, is that since my platelet is low by Hemotologist disregards my condition telling me to come back in six months and then I’m fine I’m gonna be going to a couple specialists which is great, but how do I know if my ET is advancing if the only way Dr. seem to monitor is by platelet count, I am convinced that it’s not about the count. It’s about how the platelets react in your body. If this is the case, how does one know if the disease is progressing can you tell if you are marrow is deteriorating somehow even if you have lower platelets and at what point do they put me on something other than the aspirin that I’ve been on for the last 7 years.? Other than a blood test every three months, how else can they tell any help would be so much appreciated. I feel like the doctors doctors all ignore me and are not understanding my symptoms..
ET with normal platelet count: I was diagnosed... - MPN Voice
ET with normal platelet count
You are asking a very sophisticated question with a somewhat complicated and nuanced answer. The best answer would need to come from a MPN Specialist who can review your unique presentation of a MPN.
It appears that you met diagnostic criteria for ET rather than CHIP. Given that the diagnosis was based on a BMB, I am assuming that the bone marrow morphology supported the ET diagnosis rather than CHIP. "BM biopsy showing proliferation mainly of the megakaryocyte lineage with increased numbers of enlarged, mature megakaryocytes with hyperlobulated nuclei. No significant left-shift of neutrophil granulopoiesis or erythropoiesis and very rarely minor (grade 1) increase in reticulin fibers." One way to monitor your MPN status would be a repeat BMB. A repeat BMB would only be indicated if there was a significant change in your MPN status as indicated by blood counts and symptom profile. This is an issue that would need to be discussed with a MPN expert provider.
You are right on target with the perception that ET status and symptoms are not just about how many platelets there are. It is about how the platelets behave. The erythromelalgia symptoms you describe can definitely occur without a formal erhtromeralga diagnosis. I experienced the same thing. My doc just called it "PV Toes." It does not matter what you call it. It is a significant problem no matter what you call it. Progression in this symptom would be a sign of progression in the ET. Any new or changed MPN constitutional/secondary symptoms would also be a sign of progression.
It might be worth discussing tracking inflammatory markers since they have a direct impact on MPN symptoms and risk of disease progression. Note that this is not a standard practice at this point, though some docs check CRP and ESR . There are great many inflammatory markers, including cytokines, interleukins, TNF-a, and more. The complexity of tracking inflammatory process makes it a difficult biomarker to nail down. While I have flagged high on a few of these markers, we do not regularly track them. I use my inflammatory symptoms (e.g., joint pain) for tracking since it is the actual symptoms that impact quality of life. Some MPN Specialists are particularly interested in the issue of MPN inflammation. One of these docs would give the best advice on this topic.
My PLTs used to vary between the 500s-700s. It is normal for PLT to vary as much as 100 in a single day based on what is going on in the body. If your case of ET only shows a very low level of thrombocytosis, it is possible that you would fluctuate above and below the line. It is the trend over time that matters. If your PLTs continue to trend downward, that would be a change in ET status that would merit further investigation. If you moved closer to thrombocytopenia, that would be a potential sign of bone marrow dysfunction and increasing fibrosis would need to be ruled out. This is a topic for review with a MPN Specialist.
You are absolutely correct that PLT numbers are only one element of tracking ET status. There is much more to tracking and managing a MPN. Wishing you success in your quest to get clear answers.
thank you so much for your respond was so scary is that the doctors are only looking at my platelet count and meanwhile I could be progressing and no one would know are you saying the only way to really understand if I’m progressing is just by my own analysis of Inflammation? My platelet count has never been higher than 450 and in the last seven years it’s been hovering around 300 to 325. However, the tingling and the burning of erythremia is real. Do you know of an expert I’ll fly anywhere to see anyone if anyone has someone that is really Advanced with understanding a myalgia and all the secondary symptoms of ET how do I know if my bone marrow is damaged or if I’m progressing I guess I will figure it out because no other doctors will
I think you need to review your case with a MPN Specialist who can best address your concerns with a case specific assessment. Here are two lists.
pvreporter.com/mpn-speciali...
The erythromelalgia or like symptoms are certainly bothersome. This can occur due to the MPN or for other reasons. My PCP says the simplest explanation is the best explanation. Given that you have a JAK2 positive ET, that is the easiest explanation. The absolute PLT numbers may have little to do with this issue. You are correct that it is about how the PLTs behave, not how many of them there are. Some people find that increasing the aspirin to 2x/day or an alternative antiplatelet medication works better. I would not recommend changing the intervention without consultation with a medical professional.
The only way to know if there has been progression in how the disease affects your bone marrow is to do a repeat BMB. An issue to look for would be an increase in bone marrow fibrosis. I do not know that your current situation justifies a repat BMB. That is an issue to discuss with a MPN Specialist.
There is more to progression than the numbers on a CBC. While these numbers certainly matter, there are other factors to consider. I listed some of the things I feel are worth noting but you would need to review your symptoms with a provider whose expertise you trust.
I would not want to guess at whether your ET has progressed. You need to consult with a MPN Specialist for that answer. At this point, it is worthing doing whatever is necessary for this consult to relieve your mind if for no other reason. There is no purpose in needless worry. that will just stress you out. Take action and get answers. It is far better to deal with actuality rather than fear of what could be.
probably the only way to get a exact explanation is to get another BMB and get the slides looked at my a MPN expert, 2018 is 7 years ago so maybe worth a fresh look
Platelet count
High Platelet Diagnosis Update….
