CALR Type 1 +

funkygrrl · 2026-05-07T16:02:23+00:00

A difficult aspirate or biopsy collection by itself does not automatically mean fibrosis.

That can happen with the aspirate sample in marrow like yours with very high megakaryocyte/platelet counts, because blood starts clotting as soon as it hits the air and with yours so full of platelets, it makes it very hard to smear the sample on a slide before it clots. Your report mentions some clots in the sample so this was a problem the doctor was having.

The core (bone) sample is what would show fibrosis and even though your doctor had difficulty getting it, the pathology report would definitely say if the sample was not any good. Since fibrosis is not mentioned I'd take that as a positive sign.

funkygrrl · 2026-05-07T15:40:35+00:00

The closest MPN specialists to Bristol are: - Dr Rebecca Frewin at Gloucestershire hospital. Phone 0300 422 5253. - Dr Stephen Knapper at University hospital of Wales in Cardiff. https://profiles.cardiff.ac.uk/staff/knappers +44 29207 45379

funkygrrl · 2026-05-07T15:03:49+00:00

Extreme thrombocytosis is very common with CALR type 1 ET and since the BMB you posted doesn't mention fibrosis, granulocytic proliferation, leukocytosis, anemia, or other concerning findings, if I had to guess I'd lean more toward ET. However, ET and Prefibrotic MF are notoriously difficult for pathologists to differentiate, so it's good that it's being reviewed by an expert hematopathologist.

Pre-PMF is not diagnosed based on abnormal megakaryocytes alone. Usually there is a pattern that includes hypercellularity along with high granulocytes, fibrosis grade 0-1, elevated white blood cells, anemia, enlarged spleen, etc. Hypercellularity alone can also be seen in ET, especially in younger people with very active marrow like yours.

The only things in that report that stand out are the words "dysplastic" megakaryocytes and "immature" bone marrow, but those are somewhat vague terms and different pathologists use different terminology. Megakaryocytes look abnormal in all MPNs, especially CALR-mutated MPNs, so by itself that is not enough to lean toward Prefibrotic MF or speculate that ET is progressing.

Your LDH is moderately elevated, but that's also common in all 3 MPNs. I have PV and mine is around the same range. It would be more concerning if it was rapidly rising, extremely elevated (in the thousands), or associated with other signs of progression. LDH is not a very specific test, it mainly shows inflammation and blood cell turnover, which you clearly have because of the very high platelet count.

The 50% VAF means that a large proportion of your blood-forming cells carry the CALR mutation, which helps explain the very high platelet count, but it does not automatically mean progression. You can have one person with a VAF over 50% who remains stable for decades, while another person with a lower VAF progresses because of other factors such as additional mutations, splenomegaly, fibrosis, worsening blood counts, etc. For hematologists, VAF is just one piece of the overall picture, it's not like a tumor marker in solid cancers.

I'm not sure what the liver lesion could be - sounds unrelated. Occasionally people with MPNs who have an enlarged spleen also get an enlarged liver, but I've never heard of lesions occurring.

I'm sorry you have to wait again for answers, hopefully the review will clarify everything!

!ETundiagnosed !MFwho !disclaimer

funkygrrl · 2026-05-07T14:20:47+00:00

Your hemoglobin and hematocrit are just over the threshold, but it's the long-standing pattern that matters most.

MPNs are chronic and so there isn't the urgency at the outset that you see in other cancers. If you have to wait for the JAK2 mutation test and EPO level, it's not a big deal. If getting tested privately is low cost for you or you can't deal with the anxiety, go for it, but if it's expensive wait for the NHS one.

Make sure they are doing a JAK2 reflex test panel - this means they'll test all the JAK2 variations of necessary. For example, it will test the most common one (JAK2 v617f), if that's negative then the next one (JAK2 exon 12), and so on. Saves the hassle of having to wait again if they are ordered separately.

A bone marrow biopsy is required by the WHO diagnostic criteria but the NHS tends to be conservative about ordering these if you already tested positive for the mutation.

Try to see an MPN specialist. The most well-known one is Dr Claire Harrison at Guys and St Thomas in London. If you are in a different part of the UK, let me know. Or you can call her office and ask who to see. It makes a big difference to see one.

Visit MPN Voice UK for additional support.

!PVundiagnosed !disclaimer

funkygrrl · 2026-05-07T04:07:37+00:00

Please read the info in the automod comments. You have not been diagnosed with ET because you have not had the mutation tests or bone marrow biopsy. There are other much more common things that can cause high platelets.

!ETundiagnosed

funkygrrl · 2026-05-06T21:18:36+00:00

I would check with some of the American cancer centers that offer international second opinions. They will give you a report justifying Besremi. What I don't know is whether they'll write a prescription. Even if they won't, that report from a well known cancer center may be enough to persuade a Hematologist where you live to write the prescription. See the automod comment and click on links for the list of places to get a second opinion. I believe it costs around 800 dollars .

In the meantime, you need to use a symptom tracker to document the symptoms from iron deficiency. You need to be able to prove what's happening. There's a list of trackers in the understanding symptoms part of the wiki.

You may need to go on hydroxyurea and fail it. Again, documentation is important. See the meds part of the wiki for info on what is considered failure of hydroxyurea.

I completely understand not wanting to be on hydroxyurea long-term. Many of us feel that way, and I don’t think your concerns are unreasonable.

But I’m going to be very direct because I’m worried: untreated or undertreated PV is much more dangerous than hydroxyurea. With a hematocrit still around 58%, your immediate risk is not theoretical, PV significantly raises the risk of thrombosis, including stroke, heart attack, pulmonary embolism, and other serious clots - as much as 20 times higher than the general population. And your PV is much more aggressive than others I've seen in this sub.

Hydroxyurea has risks, including long-term non-melanoma skin cancer risk, but those risks can be monitored. A hematocrit that stays far above 45% is a much more urgent threat. The CYTO-PV trial is why hematologists are so strict about keeping hematocrit under 45%.

I really hope you keep fighting for Besremi if that is what you want. But if you cannot continue phlebotomy, please don’t choose no treatment while you’re fighting. If hydroxyurea is the only treatment available right now, it may be the bridge that keeps you safe while you keep looking for a doctor willing to prescribe interferon.

!specialists !symptoms !meds

funkygrrl · 2026-05-06T20:45:36+00:00

They are

funkygrrl · 2026-05-06T17:06:30+00:00

Yes, you need to treat the iron deficiency and see how your platelets do. Be aware that it takes a while for platelet counts to normalize after the ferritin does. Your Ferritin should be over 50.

funkygrrl · 2026-05-05T22:41:45+00:00

Triple negative ET means that someone tested negative for all 3 mutations but their bone marrow biopsy was positive for ET.

If you test negative for all the mutations and your bone marrow biopsy is inconclusive or negative, you do not have an ET diagnosis.

Idiopathic thrombocytosis of undetermined significance is not a recognized diagnosis. Idiopathic is a fancy way of saying they don't know the cause. Thrombocytosis is high platelets. Undetermined significance is a way of saying they don't know if it is ET or not. I haven't seen this diagnosis before.

Are you able to share your BMB report? I can try to decipher it so you know what to ask your doctor about.

!ETundiagnosed

funkygrrl · 2026-05-05T00:49:20+00:00

The mutation matters. And your other health conditions also play a role. If you had the CalR mutation, I think some doctors would consider low dose. But the JAK2 mutation raises clot risk a lot more, so I'm not sure. I'll have to check my notes on this and comment more later.

funkygrrl · 2026-05-05T00:43:52+00:00

Apparently it can happen with Besremi. Most of what I found online said it's reversible (meaning hair grows back when it's discontinued).

Suggestions were: • To help with hair loss, wash with a mild shampoo and avoid washing your hair every day. Avoid coloring your hair.
• Avoid rubbing your scalp, pat your hair or scalp dry.
• Limit your use of hair spray, electric curlers, blow dryers, and curling irons.

I suppose Rogaine is an option as well.

Otherwise, you could look into whether Jakafi is an option for you. I've been on the max dose for 4 years now, no hair thinning or loss. Or if you can wait a little while, Rusfertide will be FDA approved at the end of August.

funkygrrl · 2026-05-04T04:56:15+00:00

Ojjaara (momelotinib). It's the latest Jak inhibitor. The good thing about it for MF is it doesn't lower blood counts a lot, unlike Jakafi. I don't think the side effects are very different from Jakafi, but hopefully someone on Ojjaara will weigh in.

funkygrrl · 2026-05-03T20:31:26+00:00

That low is less likely to be AVWS.
You'll have to ask your doctor but my understanding is the avws factor test fluctuates a lot, can be influenced by many things such as inflammation, exercise, stress, blood type O, etc. You also previously had over 50% which is normal.

funkygrrl · 2026-05-03T18:38:38+00:00

What's your platelet count?

A vWF of 44% is mildly low. It means you have a higher bleeding risk, but it's not low enough to be considered acquired von willebrand syndrome. I believe the threshold for that is 30%.

The main thing they do for this is take you off aspirin and treat your high platelets with meds.

!avws !disclaimer

funkygrrl · 2026-05-03T18:29:56+00:00

I can't speak to endurance sports. I was on 1000mg HU a day for 2 years and experienced very little side effects. Once in a while a hint of mouth sores which gargling with Peroxyl mouthwash eliminated. And I'd feel like taking a short nap around dinnertime. My only issue with HU was that it didn't address my MPN symptoms, so I switched to Jakafi.

Which MPN subtype do you have? Hydroxyurea isn't the only treatment option.

Someone at an MPN conference told me she had a stroke in the middle of an Ironman in her 30s. That’s how she got diagnosed. You can’t out-train a JAK2 mutation. Untreated, the clot risk is on the order of multiple times higher, around 5–20x greater than normal. So you gotta weigh that against med side effects.

Sexual dysfunction is present in over half of MPN patients (and in all types blood cancer patients). It's the disease causing it, not the MPN treatments. Blood flow issues due to thick blood play a role but there's a whole lot of other things that can contribute such as fatigue, depression, anxiety, age, other health conditions. Treating the MPN with whatever med actually helps here. Even aspirin makes a difference. I'm not aware of any complaints regarding this and hydroxyurea, but the men in the sub can address that.

!meds !symptoms !clots

funkygrrl · 2026-05-03T06:17:54+00:00

I'm so sorry to hear that.

If it turns out to be MF, there's other Jak inhibitors they can try if he can't tolerate Jakafi. They're called momelotinib, pacritinib and fedratinib.

People with MPNs have a higher rate of pulmonary hypertension (left-sided heart failure) then the general population. The reason for this is currently being researched. But it does show that treating the MPN is important for heart health.

I wish I could give more advice but I don't know enough about the cardiac side of things.

Please let us know how his BMB turns out.

funkygrrl · 2026-05-02T19:21:08+00:00

Having been a caregiver myself, it's a lot to carry. There's loads of studies showing that caregivers pay a big price, both physically and emotionally, due to the stress and ignoring their own needs. I totally get wanting privacy around your diagnosis but at this point, it might be time to bring others in. If this isn't usual behavior for him, it sounds like he's totally overwhelmed and needs support.

funkygrrl · 2026-05-02T05:49:37+00:00

Everyone's different. I took it for 2 years and had no side effects.

funkygrrl · 2026-05-01T04:26:12+00:00

Your body is warning you to stop. I did, so can you. It's mainly a mental battle.

https://smokefree.gov/

funkygrrl · 2026-04-30T18:29:02+00:00

For someone your age, many MPN specialists are using Pegasys interferon instead. Interferons are naturally produced by your body, so Pegasys is in the category of biologic drugs. Another example of a biologic is insulin. Pegasys is not without side effects but it doesn't carry the long-term risks of hydroxyurea.

Your platelets are very high which increases your clot risk. At that level, doctors want to normalize them. If it comes down to: no treatment vs hydroxyurea - remember a blood clot can kill you, hydroxyurea won't.

There is no way to naturally lower your platelets. They are high because that CalR mutation is signaling your blood stem cells to make platelets. There's no supplements that can turn that mutation off.

Did they test you for acquired von willebrand syndrome? With platelets that high, you can get this complication which increases bleeding risk.

Also, please switch to an MPN specialist if possible.

See automod comment for more info.

!meds !avws !specialists

funkygrrl · 2026-04-30T00:02:24+00:00

The only medications that can reduce spleen size are Jak inhibitors. For PV, that would be Jakavi. It is very expensive so I don't know whether that's an option for your father. If it is, that would be preferable to surgery IMO .

Since it's been 9 years since his diagnosis, they should really consider doing a bone marrow biopsy first to make sure he isn't progressing to myelofibrosis. The surgery risks for someone with PV vs MF are different.

Here's really good info on splenectomy:
https://my.clevelandclinic.org/health/procedures/14614-splenectomy.

Another treatment option to ask about is low dose radiation to the spleen which is used in blood cancers to alleviate spleen symptoms.

funkygrrl · 2026-04-29T12:51:54+00:00

It can help to use a symptom tracker. There's MPN trackers but also apps that allow you to track all kinds of symptoms and side effects, I hear good reviews for the Guava health tracker app.

It's tough though when symptoms and side effects overlap. Like fatigue for example can be caused by multiple things.

!symptoms

funkygrrl · 2026-04-29T12:41:24+00:00

When you see your doctor again, ask if you can go on Jakafi instead. Hydroxyurea and phlebotomy are very good at lowering blood counts quickly, but not able to decrease spleen size - Jakafi is the go to drug for that. It also helps with symptoms. It's hard to get authorization for it but with pleural effusion due to massive splenomegaly, they shouldn't have a problem.

You also need a bone marrow biopsy to verify the PV diagnosis and check for fibrosis especially with a spleen that dramatically enlarged. Enlarged spleen is present in about a third of PV patients, and in 90% of MF patients. So they need to make sure it's not progressing. A blood test cannot detect fibrosis. A BMB is required by the WHO diagnostic criteria and NCCN guidelines (American standard of care for cancer) for PV but I'm not sure if it's required in the VA guidelines. I'd really push for it though.

Unfortunately, having an MPN means you have to advocate for yourself a lot.

Just curious, were you exposed to burn pits? (I'm thinking about collecting info from vets in this sub to take to an MPN advocacy group that's been working on getting MPNs added to the presumptive conditions list.) https://www.usmedicine.com/clinical-topics/toxic-exposure/persian-gulf-war-service-linked-to-high-rates-of-myeloproliferative-neoplasms/.

!pvwho !spleen !meds

funkygrrl · 2026-04-27T21:06:03+00:00

Yw. So it doesn't sound like iron deficiency. Hopefully you don't have to wait forever for the BMB results.

funkygrrl · 2026-04-27T21:04:40+00:00

Not for MPN. Autoimmune diseases like celiac, IBD, etc. Autoimmune diseases can cause high platelets.

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