I was diagnosed with PV, JaK2+ in 2014; coming up on my 10-year anniversary soon. My treatment consisted of phlebs, ASA, and HU (for the past 18 months at 1000mg 5x/wk & 1500mg twice a week). Overall, I have been very stable for the past 2+years requiring no phlebs, except platelets still being elevated between the 6-700's, however, totally asymptomatic and being able to do all my activities & climbing mountains.
I see a local heme/onc every 6 months with monthly CBC's performed and my MPN specialist at MSKCC annually, mostly through telemedicine since I have been doing so well.
I had genetic testing this year through MSK & had developed 3 non-driver mutations, TET2 5%, DNMT3A 4%, SH2B3 2% and my driver mutation Jak2 at 58%. Since I was doing so well, no change in medication was warranted.
Last week I received my CBC in my LabCorp portal and was shocked to see that all my blood levels had tanked. I wasn't noticing any new symptoms except that I would doze on & off at night for the past month while watching TV, which I have never done before, but figured it was my active lifestyle & aging (I am an active 63 yr old)
When I look back at the trends of my bloodwork, I have slowly been trending downward since April of 2024, but with no symptoms & nothing that my local heme & MPN specialist picked up on or had concerns. This last blood test had significantly dropped from the one the month before. Since it was one major drop, I went into my local heme to ask for a repeat blood test to rule out an anomaly. That was last Monday & it confirmed my fears...WBC at 1.8, RBC 2.48, HBG 9.3, HCT 29.3 and platelets dropped in one week from 619 to 494. Therefore, I am being scheduled for a bone marrow biopsy this week at a local hospital. Since I am so anemic & neutropenic, going down to MSK on public transportation would not be a viable option. However, once I do have my pathology report, I will confer with my MPN specialist for my treatment. I had asked what to do about taking HU in the meantime, & I dropped it down to one pill a day since my blood levels may continue to drop weekly & I will require a blood transfusion since no injections are recommended until after my biopsy. I find it hard to believe that things could change so dramatically in such a short period of time. I now have very little energy, the start of early satiety (even though I cannot palpate or have ever had an enlarged spleen) with some new joint pains. I still do my yoga and gentle pilates, however, I cannot tolerate the high intensity workouts like I did just over a week ago. I am a retired physical therapist, so exercise has always been an important part of my life. But I know my limitations too, now that I am so anemic. I now monitor my vital signs, especially while working out.
Has anyone else had these types of issues happen so quickly? I have seen no Blasts or absolute NRBC on my last lab report. I am in between knowing what is happening, (although looking like postPV-MF) hopefully not AML, and might take a few weeks to receive the pathology report once completed. My concern is what to do about my current meds, (is dropping it to 7 pills a week enough?) Since it happened on a holiday week when no one is available, just a 15 min telephone conversation with my local heme, who I have had to suggest decreasing my HU dosage, increasing blood testing, etc. He recommended the BMB, which I knew was imminent. Any advice while I await results? I will, of course, contact my MPN specialist again, (l have kept him updated through my patient portal) but not sure how soon I will be able to travel the 3 hrs to see him in person, under these circumstances & my very low energy level & the need to stay as healthy as possible & not pick up any viruses this time of year that may be difficult for me to fight off. It might have to be a Zoom telemedicine visit until things improve?!
Thank you and looking forward to some advice from this list of very smart, caring people who understand what I am currently going through.
Donna
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ADKlover61
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Sorry to hear about this change in your PV status. It is hard to say what is going on as you will need the BMB results to know. It may be that an even lower dose of HU is indicated since the med can itself cause anemia and other cytopenias. Your MPN specialist is the best source for this advice. It may also be that you need a change in medication. Besremi or Jakafi may be a better choice even if you have not progressed to MF, particularly in light of the presence of non-driver mutations. I also have a non-driver mutation present (NF1), which is part of why I opted for Besremi in hope of reducing risk of progression. Thus far, Besremi has reduced my JAK2VAF from 38% to 10%. I am rechecking the VAF tomorrow and hope for good news.
I would check with your MPN Specialist about your level of neutropenia. My MPN care team feels that provided my NEUT > 1.00 and LYMPH > 0.50 then my immune system would still respond adequately even though I may be below reference range. From occasional infections I would say this seems to be true.
Please do let us know how you get on. All the best.
Thank you for your quick reply Hunter (Steve). It was encouraging to say the least. I had never thought that possibly the larger dosage of HU I have been taking for the past year in a half might be part of the cause for the drop in levels over time- that would be great if that were the case. As you mentioned, I will not know that until I get the results of my BMB. Since I haven’t spoken directly with my MPN specialist, it will be interesting to hear his thoughts since I just had my appt with him one month ago & I was doing great. When I asked about changing meds due to my high Jak2 VAF and new non driver mutations, he did mention that since I have dual Jak2 & DNMT3A mutations, that the interferons do not work as effectively. He mentioned that Jakafi is showing less thrombotic risk, decreased risk of progression and improving VAF over HU, however, since I was doing so well on current treatment, that we didn’t need to change unless I was requiring an increased dosage to remain stable or a change in symptoms, etc. This certainly falls in those categories!
My absolute NEUT are currently 0.98 and LYMPH 0.73 at present. I have had no infections or even a cold in quite some time, years actually, so hopefully you are correct, although I am going back to wearing a mask & avoiding large crowds while it is at this level just to be safe.
It is encouraging to see how well you have done since switching to Besremi. Good luck on your VAF test tomorrow. Thanks again for your support & I will update once I have more info & pathology report.
Hi there, I am sorry to hear that you have become so unwell so quickly. The best option is of course the BMB, but the HU could be contributing towards you lowered blood counts. I took HU over a period of a few years and it eventually pushed me into neutropenia and anaemia. I had a BMB which has shown progression to MF and I am now on peg interferon. Without the drop in bloods I would not have had the BMB and be on what I consider a better medication. I really hope that you get answers quickly. All the best. Penny
I’m sorry to hear you’ve had such an abrupt decrease in your CBC values. I hope you can have the BMB quickly in order to assess what is happening. I suspect your hematologist might recommend holding the HU until they have a clearer picture of what is going on. I will be thinking of you and hoping for a quick and thorough assessment and treatment plan. Please keep us updated.
I'm so sorry Donna to hear about your alarming transformation in your health. My mom had CML, she made her 10 year with just close monitoring for the first 9 years or so, then as her platelets reached the million mark her oncologist decided to put her on HU. I don't recall her daily dose, but after about a year on it, her doctor increased her dose (in spite of her bloodwork looking amazing - normal even). 4 months after the dose increase she started feeling rapidly exhausted and could barely walk around the block. She revisited her oncologist in May of 2020, was rushed for a BMB and the worst fear came to fruition. Her CML had changed to AML. My mother was 66 and died 7 weeks later.Definitely please seek medical attention ASAP. I don't want to make you feel more frightened than I'm sure you already are. I wish for you that your story has a much happier ending than my mom's did. It sounds like you have a great support team on your side. Wishing you all the best and hugs from Canada.
Since you joined the forum 9 years ago you may have read many posts about interferon being a drug that often slows or halts disease progression, especially when started at the time ET and PV is diagnosed.
MSK, unfortunately, does not have a track record of commonly offering interferon to ET and PV patients for disease progression suppression purposes, whereas Weill-Cornell, a few blocks away in NYC, does.
If your upcoming BMB reveals progression to post PV MF has occurred it may be too late for interferon to highly helpful although it still might if combined with a hemoglobin boosting drug.
If you want to pursue the possibility of trying interferon + hemoglobin booster I'd suggest considering making an appointment with the interferon experts at the Silver MPN Center, at Weill Cornell Medicine in NYC, e.g. with Ghaith Abu-Zeinah, M.D. (646)962-2727. MSK doesn't have any interferon experts.
so sorry to hear of your dip in blood results. I cannot help you. So just letting you know I’m sending hugs. All the best for your forth coming procedure xx
sorry to read about your concerns, it’s great your getting a BMB so quickly to check.
Re the dose of HU, I wonder why take any HU currently if your counts appear to have fallen so quickly , that’s one to check with your specialist though. The platelet drop in a week isn’t in itself too significant, platelets can jump around by 100-200 for no great reason. Platelets under 1 million are not usually treated by experts unless symptomatic or other risk factors.
You don’t say what your previous dose of HU was, hence harder to say how much the dose increase potentially played a part in count drops, also you didn’t say what your previous counts were for Hgb, Hct, whites etc.
So it’s all a bit speculative until the BMB is done.
One clear thing is it’s wise to discuss all this with your MPN specialist asap and if need be a second opinion from another MPN expert. We can give bits of opinions on here but only a real MPN expert can advise you on the haematological issues.
In the meantime if it were me I would be asking why not reduce the HU further and monitor weekly your counts.
As you may be aware HU doesn’t do anything for potentially slowing the disease or lowering allele burden in the long term, the other meds might for some patients ie Peg/Bes or Rux. Again depending on the outcome of the BMB that would also be something to consider.
We look forward to hearing how you get on and fingers crossed in the meantime.
I wanted to send a quick note to wish you luck with your upcoming appointments to figure out what is causing these changes. Like Hunter(Steve), I suggest you ask about Besremi, which has been effective for me. Please keep us posted.
This is why the some doctors think it, but the reality is very diferent. When you have this gene, DNMT3A, you must take Besremi in low doses, 100 or 150mcg starting with 50mcgr. The answer the body is good. This paper is what too much hematologists think, but there are others dont think so. Personalitation is the way. pubmed.ncbi.nlm.nih.gov/384...
I was very fortunate to have started Besremi from the start of any treatment. I am pleased to say I have been staying very healthy and my numbers have been excellent. My JAK2VAF before treatment was 78% 22 month re check I'm 7%. I hope you find a solution quickly so you feel better. Keep on pushing my friend. May I add Hunter is a wonderful asset to this community he has helped me all along my journey.
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