Full blown PV now

[Lena70]

I was diagnosed with Jak2+ PV back in January 2020. At that time, I didn't have high RBC, Hemaglobin, or hematocrit. I had high platelets, high WBC, basophilia, and eosinophilia. My hematologist said she was calling it PV rather than ET due to the high counts other than my platelets.

She was completely correct. I've been unmasked, so to speak. We're still watching and waiting. I kind of question why when my hematocrit is at 44.5, but I figure we'll hash it out on the next appointment. She still considers me low-risk due to my age (52) and fitness level and weight.

If you recall, my MPN was missed for seven years. I started with high platelets then weird and wonky white blood counts, and now I'm here. It took ten years to go from ET to masked PV to PV.

Anyone else have a similar trajectory? I know Hunter progressed from ET to PV, but can't recall the timeline.

[EPguy]

I have PV Dx with ET features. My non-PLT counts were reasonably behaved without meds and BMB suggested ET. But enough ambiguity to give the PV Dx.

According to the familiar 2016 WHO criteria, you don't meet the requirements for PV which calls for HCT >48.

table 1 in- ncbi.nlm.nih.gov/pmc/articl...

Doesn't mean you don't have it, but could be one reason your Dr is on the waiting agenda. A BMB could provide more insight.

One advantage of a PV Dx is on-label access to Besremi and Jakafi (Rux).

[monarch5000]

At 52, you may be considered at low risk of dying prematurely from a blood clot, but you are not necessarily considered low risk of dying prematurely from disease progression to myelofibrosis. Dr. Silver explains in this short video: youtu.be/JXI9FwRcauw(Not working as expected?)

[ainslie in reply to monarch5000]

it’s important to remember the majority will not develop mylofibrosis, that Wąs confirmed in a recent consult with Dr V, it’s also important to note the graphs in Dr Silvers presentation state it refers to high risk patients, Lena may not be high risk.

[Lena70 in reply to ainslie]

My doctor considers me low-risk right now due to age and my weight and fitness level.

[Timjonze]

yes I’ve had the same trajectory. Labelled ET and then PV but I see a different doctor every time and one of them always referred to it as PV rather than ET despite the only abnormal result being slightly high platelets. Now my platelets have stayed stable (even dip into normal range sometimes) but my haematocrit hit 0.52 and white cells are raised. I’m currently on venesections which has kept everything low (although slightly low iron now so can get tired some days). I feel like the ET and PV distinction is becoming a little less relevant for clinicians compared to the genetic mutation involved and speed of changes in the blood. Good luck with everything

[Lena70 in reply to Timjonze]

Thank you! Same with you.

[STK52]

Hi Lena

I’ve a similar story. My platelet was high (>550) which triggered an investigation last year. Eventually, my hematocrits caught up and went over 55. I was diagnosed formally after BMB with PV. I’m also 52 and manage at the moment with venesection and aspirin. My platelet is still over 800 which I’m a little concerned. What symptoms are you getting?

[Lena70 in reply to STK52]

My main symptom is fatigue, but I found a solution to that. I started taking Wellbutrin 150XL and it has given me the pep I had lost. Most of the symptoms I had before diagnosis were solved with my daily aspirin. I had terrible headaches. I also had problems with itchiness, but a daily antihistamine and Pepcid have helped greatly with this.

[hunter5582]

You are correct about my progressing from ET to PV. I was diagnosed with ET about 31 years ago. It progressed to PV about 9 years ago, when the erythrocytosis became evident. I also experienced mild basophilia. At the time of the progresion, I experienced a case of gastroparesis that lasted about a week. That caused significant gastritis and reactive thrombocytosis. I do not think that this is a coincidence. I know that at the time my cortisol levels were also high. Had we checked, I am sure a number of inflammatory cytokines would have been elevated.

The hematologist I was seeing at the time missed the progression. I did not realize that I had progressed until 5 years later. When it became evident that he misdiagnosed me, that doc fired himself. He said "Your health is more important than my ego." I now have a MPN care team that includes a wonderful local hematologist and a MPN Specialist who consults on my care plan.

MPNs can evolve and change over time. Preventing progression is an important treatment goal. We are fortunate that it may be that is an option for us due to the progress being made in treatment.

All the best.

[GardNerd]

Reflecting on EPGuy’s comment and the video posted by Monarch, I do think there’s a good argument for doing a BMB to get a for sure diagnosis. If it is PV for sure, you could talk to your doctor about getting on an interferon. The Silver video talks about the benefits of interferon for both high and low risk PV. His concluding statement is that patients should consider an interferon early in the disease (I’m paraphrasing here).

My one regret in my disease process is that I didn’t get on an interferon earlier. I was fit and healthy, cruising along with few symptoms and periodic phlebotomies from age 44 to age 60. At age 60, I got my second BMB (first one at diagnosis at 44). Turns out, things were progressing all along — I just didn’t know it. Now I feel like I’m playing MF defense and maybe an interferon could have slowed things down during that entire period.

There are several treatments now available and on the horizon, so it feels like there’s benefit to slowing this thing down while we go out and live our full lives.

Best to you!

[Xuzy in reply to GardNerd]

What grade fibrosis was noted in ur bmb? I heard sometimes interferon or jakavi reversed fibrosis

[GardNerd in reply to Xuzy]

I think it was at level 2 when I got the BMB about a year ago. They're going to do another one here pretty soon, which will be the first one after being on Besremi. My fingers are crossed that the allele burden is coming down. Cuz it was pretty darn high.

[Meatloaf9 in reply to GardNerd]

Hi, was your diagnosis changed to MF a year ago when you got the BMB?

Do you know what your AB was before and after the BMB?

Best to you on your next BMB, please let us all know how it goes. Hoping you get a good result.

[GardNerd in reply to Meatloaf9]

I still have PV fortunately. I feel really good (always have since diagnosis) and am hoping for little to no progression since my last BMB. I don’t even want to put the allele burden in writing — makes it too real, I guess. But it was closer to 100 than 50. I’m working on living life every day and trying not to worry about the future any sooner than necessary. 😊

[EPguy in reply to GardNerd]

GardNerd:

With VAF at that level it's very likely homozygous. This is discussed in some older posts; it should respond nicely to IFN to at least something pleasantly lower. No guarantees of course for any of us.

[Meatloaf9 in reply to GardNerd]

Good luck with Interferon. I think I remember someone posting on here that their AB went from the low 80's to the teens. Hoping you do at least that well. Best

[Xuzy in reply to GardNerd]

Wishing you the best and hoping your fibrosis levels are back to zero again!! Or lower.

[GardNerd in reply to Xuzy]

From your lips to the ears of whoever is in charge! 😊

[MAP44]

Hi Lena70,

Funny I was diagnosed at age 52 (in 2020) with ET from a BMB, Jak2. Platelets were high and I was feeling crushed physically. Started on the aspirin and felt so much better. Only months later my hematocrit started to climb quickly and again I was exhausted. Unmasked PV begins. I had a few months of phlebotomy every 3 weeks and I started feeling better. I was fortunate to be able to start Pegasys a year ago and what a wonderful experience, no more phlebotomy. I am a slower bleeder so although the phlebotomy went well it took time at the appointments.

Looking back at blood test I had high platelets 17 yrs ago after my youngest was born. Years later I moved to another city and doctor and never really had any blood tests. Yes I had Pap tests and such but no blood work taken or needed. Surprised with diagnosis but over that shock now.

🍻 Wishing you all the best.

[Lena70 in reply to MAP44]

Thanks!

My rising platelets were missed for 7 years despite yearly blood tests for my annual checkups. A pathologist flagged my CBC and smear back in 2018. My doctor at the time was having issues and was fired from the practice group and the next doc didn't notice the note. I have a very good Primary Care Doc now.

If it wasn't for my persistence and noticing my high platelets, I would have gone even longer before diagnosis. I mentioned the platelets to my Rheumatologist. She consulted a hematologist and I was diagnosed in Jan 2020.

[MAP44]

Wow! Happy to hear you have great Doctors in your corner now. ❤️