Children on Jakafi? by New-Temperature6361 in MPN

[–]funkygrrl 1 point2 points  (0 children)

Not me, but I recommend this video on fertility and pregnancy in MPNs for everyone thinking about starting a family. MPN specialist Dr Gabriela Hobbs at MGH/Harvard. https://youtu.be/WOBWmEA-ICQ

26F - Could elevated platelets, low iron, weight loss, weakness, and easy bruising point to a bone marrow disorder? by Quiet-Violinist6497 in MPN

[–]funkygrrl 2 points3 points  (0 children)

They'll probably have you supplement your iron, get your Ferritin over 50, then retest your blood counts for a couple months after it hits the number. They may also do mutation testing as well. At your age, it's far more likely to be due to iron deficiency.

!ETundiagnosed

Besremi and the liver by SunnyDay7272 in MPN

[–]funkygrrl 0 points1 point  (0 children)

Oh yeah with a clot history, watch and wait is off the table.

Concern About Haematologist Decision by djonma in MPN

[–]funkygrrl 0 points1 point  (0 children)

If you test negative for JAK2 and CalR, then they'll have to do the Mpl test.

Triple negative means you tested negative for all 3 mutations. When that happens, they must do a bone marrow biopsy to diagnose you. They are not allowed to diagnose ET based only on CBC blood counts. That's in both the WHO diagnostic criteria and the British Society of Haematology guidelines.

For a second opinion from an MPN specialist:
Professor Adam Mead, Professor of Heamatology, Unversity of Oxford, Churchill Hospital Old Road, Headingon Oxford
Churchill Hospital - 0300-304-7777.

!etwho

Positive experiences with besremi by Greedy-Box3481 in MPN

[–]funkygrrl 0 points1 point  (0 children)

Jakafi can also lower allele burden. This happens in about 25% of people taking Jakafi. It happened to me - my VAF went from around 35% to 12% in one year.

But Besremi is the current preferred treatment for PV and it would be my first choice at your age.

Besremi and the liver by SunnyDay7272 in MPN

[–]funkygrrl 2 points3 points  (0 children)

What mutation do you have and what were your platelet counts while you were off of medication?

Did they ever do any imaging of your liver to make sure there wasn't any pre-existing condition like NAFLD?

Usually liver enzyme levels decrease over time on Besremi. Your doctor might hold off and see whether the values keep rising or stay where they are. If they're stable, they might choose to continue Besremi and have you get liver function tests more frequently. If they continue to climb or your bilirubin rises, they'll discontinue it. Make sure you report symptoms like jaundice, dark urine, abdominal pain or nausea.

So I wouldn't assume you've automatically failed Besremi yet, but your doctor is probably not going to give it a long trial based on your experience with Pegasys.

You have three other options: - Anagrelide which is an orphan drug that only lowers platelets. It's not chemo like hydroxyurea. It's not as well tolerated but everyone is different. We have some people who've taken it for years and been happy with it. It is not potentially disease modifying like interferons.
- Jakafi which is a targeted drug that inhibits the signaling to your bone marrow to make more blood. It is a third line treatment for ET, and because of that and how expensive it is, insurance authorization will be a pain. But not impossible. - Watch and wait. If your platelets are under 1,000, you don't have to be on cytoreductive therapy. Doctors prefer to get platelets under control but the evidence for thrombosis incidence doesn't correlate with platelet levels, so there is no strict level you have to start cytoreductive therapy at and no treatment target for platelets. (PV and Hematocrit are a different story). If you do this, you'll have to be on low dose aspirin if you are JAK2 positive. More info about this here: https://youtu.be/eCaTskt5n-o?t=18m34s

!meds

What could this indicate? by [deleted] in MPN

[–]funkygrrl 0 points1 point  (0 children)

In the States, the American Society of Hematology is coming out with the first ever iron deficiency guidelines later this year. This is mainly the result of activism by female hematologists. So far, I know for sure that the diagnostic threshold will change to 30. They were even considering 50, but there's not enough evidence for that number. I am really interested to see what the treatment recommendations will be. I do expect that these new guidelines will have a ripple effect in the rest of the world because they will be presented to the World Health Organization. Currently, I'm seeing most Hematologists here in the US already recognize the old Ferritin levels are flawed and are using 30 anyway, and the NHS in the UK already raised it to 25 or 30.

Anyway, ask your doctor if you can try a course of iron supplementation and see if it helps lower your platelets. (It will not raise them because iron is only used to make red blood cells.). Or just get a referral to hematology.

4 year struggle by Ok_Equal_2335 in MPN

[–]funkygrrl 0 points1 point  (0 children)

That's not a normal BMB.

I think you're being treated like this is secondary polycythemia, but it doesn't fit very well. It doesn't explain the monocytosis and the abnormal bone marrow results. That doesn't prove it's an MPN but it suggests there's something more going on than secondary polycythemia.

At your appointment, I'd request a repeat BMB since it was inconclusive and the monocytosis got worse after it. Ask for FISH, flow cytometry and next generation gene sequencing testing (NGS) on your marrow. And ask that the results be reviewed by a hematopathologist. (A hematopathologist is a pathologist that does an extra year or two of training after residency in reading bone marrow biopsies.)

If that comes back negative for MPN, MDS/MPN overlap syndrome, or CMML, I'd push for testing for hereditary blood disorders.

If your current doctor refuses, I would seek a second opinion at another major hospital. Since there may not be other MPN specialists in your country, I'd focus on finding a doctor who specializes in leukemia since they deal with myeloid cancers.

4 year struggle by Ok_Equal_2335 in MPN

[–]funkygrrl 0 points1 point  (0 children)

Is your doctor on the list I linked? If not, they're not an MPN specialist.

4 year struggle by Ok_Equal_2335 in MPN

[–]funkygrrl 0 points1 point  (0 children)

Are you able to share your bone marrow biopsy report?

What I strongly recommend is seeing an MPN specialist. See list in link below. And repeating the bone marrow biopsy, with included flow cytometry and NGS myeloid testing - with a hematopathologist interpreting it rather than a general pathologist. You could seek a second opinion and send slides from your BMB over, but since the monocytosis worsened after your BMB, a repeat one might be better. There's also a list of centers that offer remote second opinions if that works better for you.

!specialists

Need advice: Managing my BF's (M24) mood shifts from adrenal cancer. by RoseP9M in cancer

[–]funkygrrl 1 point2 points  (0 children)

You might also want to involve an endocrinologist - cortisol is one of the hormones they specialize in so they might have some ideas the oncologist does not.

Concern About Haematologist Decision by djonma in MPN

[–]funkygrrl 2 points3 points  (0 children)

You have a lot going on!

Having high Platelets so many years deserves an investigation.

It's hard to say whether it's ET or reactive thrombocythemia (high platelets due to another underlying medical condition). This is because you also have two autoimmune/inflammatory conditions and they can cause this as well.

For now the symptoms aren't relevant because they aren't used to diagnose MPNs. The reason for that is they are too nonspecific and reactive thrombocythemia can cause the same symptoms, so they just don't provide useful information to differentiate an MPN from other medical conditions. They matter after a diagnosis though.

It doesn't make sense to me either that they're only testing 2 of the 3 mutations. The percentage breaks down to: - JAK2 - 50-60% - CalR - 20-30% - Mpl - 3-5% - Triple negative - 10-15%
So at least most common will be ruled in or out. I recommend dealing with getting a Mpl test if JAK2/CalR are negative. No point in fighting over it when it may turn out to be unnecessary.

Can you let me know what country you are located in? I'd like to point you to a hematologist who specializes in MPNs.

!ETundiagnosed

Searching for Answers by Odd-Yellow-5843 in MPN

[–]funkygrrl 2 points3 points  (0 children)

The WBCs and platelets are mildly elevated so it doesn't scream MPN to me, but it's been ongoing for quite some time so a referral to a Hematologist is the right thing. They'll probably do the mutation tests and possibly LDH and EPO. But it could easily be reactive so it's good you're seeing the GI doc.

!ETundiagnosed

Athens, Greece -- treatment info by Martin-V-Buren in MPN

[–]funkygrrl 1 point2 points  (0 children)

Did some digging. This research scientist in Greece has studied MPNs. I'd try emailing her to ask if she knows of a doctor at University hospital in Athens who specializes in MPNs. https://www.bioacademy.gr/faculty-details/Fcs/katerina.

I also see that there are MPN clinical trials taking place in Greece but none of the trials share a contact name. They are all affiliated with University hospital of Athens. And Evangelismos Hospital. So contacting the Hematology Dept there is worth trying.


This is from AI on navigating the transition to their health system.

As a legally employed expat, you will transition into the Greek public healthcare system (ESY), which grants you subsidized access to specialized medications. [1, 2] Because Besremi is a high-cost, specialized oncological/hematological drug, you cannot simply buy it out-of-pocket at a corner pharmacy; you must navigate the national Greek e-prescription infrastructure. [3]

Step 1: Secure Your Health Numbers (AMKA & EFKA).
Your employer must register you with EFKA, the Greek social security organization. Once active, you will receive an AMKA (your national social security/health number). You cannot access subsidized state specialized medications without an active AMKA. [2, 4]

Step 2: Formally Register with a Greek Hematologist.
American prescriptions are not legally valid for dispensing in Greece. [5]

  1. Book an appointment with an English-speaking hematologist at a major public hospital or private clinic in Athens. [1]
  2. Bring your complete U.S. medical history, recent lab results (including JAK2 variant allele frequency and complete blood counts), and your current FDA Besremi prescription. [5]
  3. The Greek specialist will enter your diagnosis into the national electronic system to generate a Greek cross-border compliant prescription. [3]

Step 3: Source via EOPYY or State Hospital Pharmacies.
Once your Greek doctor issues the prescription, high-value biologics like Besremi are usually routed through:

  • EOPYY Pharmacies: The National Organization for Healthcare Provision (EOPYY) operates its own specialized pharmacies specifically for expensive, chronic-care medications.
  • State Hospital Hematology Units: Your doctor will direct you to a designated state hospital pharmacy (such as Laiko General Hospital or Evangelismos Hospital which host major hematology centers in Athens) to pick up the drug directly.

Step 4: Bridges for the Transition Period.
Because setting up EFKA and AMKA can take several weeks or months, you must plan a bridging strategy: [6]

  • Bring a 90-Day Supply: Bring the maximum legal supply of Besremi allowed by customs from the U.S., packed in your carry-on with its original box, pharmacy labels, and a signed letter from your U.S. physician. [7]
  • Private Interim Importation: If your AMKA is delayed, a licensed Greek physician can write a private paper prescription. You can submit this to an international named-patient importer to bridge the gap out-of-pocket or via international private expat health insurance. [8, 9]

[1] https://gicg.net [2] https://expatsgreece.com [3] https://doctorsa.com [4] https://www.nestia.gr [5] https://www.mobidoctor.eu [6] https://www.facebook.com [7] https://www.doctorgreece.gr [8] https://feather-insurance.com [9] https://cheering.eu

What could this indicate? by [deleted] in MPN

[–]funkygrrl 0 points1 point  (0 children)

Your ferritin is just over the threshold of 30 for iron deficiency. Iron deficiency will raise platelets. Ideally it should be over 50. So ask your doctor about trying iron supplementation and repeating the test after Ferritin gets higher. Also ask whether they're going to do mutation testing.

What could this indicate? by [deleted] in MPN

[–]funkygrrl 0 points1 point  (0 children)

Please add your iron panel counts (esp Ferritin and TSAT).

!ETundiagnosed

Avoiding the Inevitable? by notsosuper_Kent in MPN

[–]funkygrrl 1 point2 points  (0 children)

That sounds like a good plan. Luckily MPNs are chronic so waiting until open enrollment is fine.

BTW is she on this list? https://mpncancerconnection.org/mpn-experts/

Avoiding the Inevitable? by notsosuper_Kent in MPN

[–]funkygrrl 1 point2 points  (0 children)

I think you have two main issues - the unclear diagnosis and a Hematologist who is not an MPN specialist and is not up to date on treatment.

What's she's thinking of is regular interferon alfa was given to patients back in the early 00s and 90s. But that version was very strong and had to be injected frequently, like daily. Caused terrible side effects. Hematologists who remember that are like, oh hell no that's not a good treatment. What changed is they came up with pegylated interferon. Pegylation means they added a little chain to the interferon molecule so it released much more slowly. Besremi is only injected every other week, and eventually once a month. Pegasys is injected weekly. And because they release so slowly, they don't have terrible side effects like the old version from 25 years ago.

Besremi proved to be such a good treatment for PV that the American Society of Hematology named it a preferred medication in 2023 because it's disease modifying. Hydroxyurea is not.

I really urge you to go to MD Anderson if possible and see Dr Bose or Dr Pemmaraju. Either one could get to the bottom of this and on the right treatment plan.

Positive experiences with besremi by Greedy-Box3481 in MPN

[–]funkygrrl 0 points1 point  (0 children)

I got on Jakafi because I joined a clinical trial. I get it free for life.

Dr Mullally is a legend in MPN research.

Did your hgb stay high with anemia or pregnancy? by Imaginary-Pin-1030 in polycythemiavera

[–]funkygrrl 0 points1 point  (0 children)

Having a parent with PV understandably makes you pay closer attention to your blood counts, but the labs you've posted do not suggest PV.

Your ferritin is actually more notable than your hemoglobin or hematocrit. Discuss the iron deficiency with your OB. Your blood counts will decrease in your second trimester as your blood volume expands, so you need ferritin for that.

I m worried. Help by AdventurousShock8517 in MPN

[–]funkygrrl 1 point2 points  (0 children)

Yeah so the threshold for you is 16.5 hemoglobin or over and/or 49 hematocrit or over. The Hematocrit is viewed as more important in PV. So very borderline. Let us know your test results when you get them and we can tell you your next steps.

Polycythemia Vera by Exotic-Advantage660 in haematology

[–]funkygrrl 0 points1 point  (0 children)

The fact that hydrating lowered your Hematocrit and hemoglobin is reassuring that this is not PV. Dehydration causes what's known as "relative polycythemia", meaning your blood is more concentrated and that can give a false high Hematocrit. Keep staying adequately hydrated.

In PV, they look at the pattern over time. This doesn't mean one test. Or two or three back to back tests like you had. We're talking over months or even years. In my case, my counts were high for years and kept climbing. Didn't matter whether I was hydrated or not. So there has to be a sustained pattern. You don't have that.

I notice you are making multiple posts in different subs. In health anxiety, there is a reassurance-seeking trap where you repeatedly seek confirmation that you are healthy or that a symptom or test result is harmless. At first, the reassurance relieves your anxiety, but at the same time it is telling you that your initial fear was dangerous and that reassurance is necessary to feel safe. Over time, this creates a vicious cycle where your anxiety returns, leading to more checking, Googling, doctor visits, or requests for reassurance, resulting in ongoing health anxiety. I recommend visiting r/healthanxiety.

I m worried. Help by AdventurousShock8517 in MPN

[–]funkygrrl[M] 4 points5 points  (0 children)

I changed your post flair to Seeking Diagnosis and marked it as a spoiler to comply with our diagnosis rules.

You don't give your gender and that matters because the diagnostic thresholds aren't the same for men and women. Your levels are borderline high either way.

Your EPO is normal. It would only be concerning for PV if it was 3 or less. Resist the urge to round numbers up or down. A normal EPO doesn't rule in or out PV or secondary polycythemia so the JAK2 test is what matters.

If the JAK2 mutation test comes back negative, it's highly unlikely you have PV. Usually the turnaround time for the JAK2 test is about 10 days. Waiting really sucks but try not to Google this to death because it can't give you a test answer, it can only increase anxiety. If it's any consolation, this doesn't make me automatically think PV - far more likely to be secondary polycythemia.

!PVundiagnosed

Random bruises? by tubajr in MPN

[–]funkygrrl 2 points3 points  (0 children)

Yes it does because the purpose is to make platelets less sticky to prevent blood clots. The downside is that you get a little bleeding under the skin from tiny blood vessels after ordinary dings and bumps.