I’ve had Polycythemia Vera for 10 years. Dr. suspects it is turning into Myleofibrosis

[Cknight6]

Good morning. I was diagnosed about 10 years ago with Polycythemia Vera. I am Jak2 positive. I was prescribed hydroxyurea and one low-dose aspirin a day. I also had phlebotomies when my hematocrit was over 42. Recently my hematologist felt like my polycythemia vera May be changing into Myleofibrosis. He took me off of Hydroxyurea and stopped my phlebotomies. However, my blood counts (Red blood cells and Hematocrit) continue to increase. I also had a bone marrow biopsy that came back “unremarkable”. The doctor that did the biopsy told my family that I did have fibrosis (apparently he could tell that when performing the biopsy). I have recently started taking Jakafi. I thought my red blood cells and hematocrit would go down, not up, if I truly have Myleofibrosis.

[Rachelthepotter]

Hi. Glad you are on Jakafi, which does seem to be the best thing for both PV and MF ( which is what I have). You’ll need to talk to your doctor about what your blood counts are doing, and why. If (s)he isn’t an MPN specialist, it’s worth getting a second opinion from someone who sees a lot if MPN patients. I hope all goes well for you- do keep posting.

Rachel

[Cknight6 in reply to Rachelthepotter]

Thank you, Rachel. I’m hoping my doctor jumped the gun when telling me he thought I had myleofibrosis. It seems Polycythemia Vera and Myleofibrosis have a lot of the same symptoms. Again, thank you so much for responding to me! 😊

[Kelly2]

I am astounded reading that your doctor "felt" that you have myelofibrosis! How did he make this assumption without proof from the BMB? And changing your treatment like that?! I would go find another specialist fast to get a second opinion.

[Cknight6 in reply to Kelly2]

Thank you, Kelly2, for responding. After he took me off of Hydroxyurea, my spleen became much larger quite quickly and I lost about 15 pounds in a month. All symptoms of Myleofibrosis I think. However my blood tests didn’t seem to reflect that. Then when I had the BMB it didn’t show whatever it should show to be able to diagnose Myleofibrosis. He has since put me on Jakafi. He still is sticking to a Myleofibrosis diagnosis. I’m confused based on the BMB. I thought it would be more definitive. (Sorry this is so long 😳)

[Kelly2 in reply to Cknight6]

Ok. Spleen enlargement is some sign

[fee13]

Spleen enlargement and weight loss can occur with any of the MPN's. My concern is BMB being unremarkable, usually for MF to be diagnosed there would have to be grade 2+ fibrotic changes (maybe 3+?) but they should have determined what MPN you do have at this stage by that biopsy. Your doctor should not be treating you for something you have not got. There is always the chance the BMB was not interpreted correctly unless they really know what they're looking for.

[Cknight6 in reply to fee13]

Thank you so much for this information. It is so helpful. 😊 Most of my symptoms started when I abruptly stopped Hydroxyurea. That may have nothing to do with anything but it makes me wonder.

[Sand-Dancer]

Hi Cknight6. I was diagnosed last October with post PV MF. (I have had PV for at least sixteen years).

The reason being my three monthly blood test suddenly showed some ‘teardrop cells’. This is an indicator apparently. So maybe ask if this is what showed up in your bloods before your BMB.

I was left on the same medication as it was working for me (hydroxy and aspirin).

I hope you get some clarity soon as I understand only too well what it is like to not be in control of what is going on.

Also, as others have suggested, you need to be seen by an MPN specialist. Which area do you live? Is there a specialist close to you?

Lesley x

[Cknight6 in reply to Sand-Dancer]

Hi Leslie. Thank you for reaching out to me. That may have been what was seen. I will definitely ask about that. I live in the United States. There is a cancer center in Houston, Texas that seems to be very knowledgeable about MPN’s but they are about 1500 miles from me. I still may try to go there. I’ve been reading about stem cell transplants and I understand they do them there. Have you considered a stem cell transplant? What do you know about that?

Cindi 😊

[Sand-Dancer in reply to Cknight6]

Hi Cindi. I am so lucky as I am now at a hospital which has a great MPN facility.

I did ask about stem cell transplant and the donor register. I was told it was too early to think about that as my fibrosis is minimal at the moment. Also I am over sixty so too early could also mean nothing as there seems to be an age restriction on it😊

[Meatloaf9 in reply to Cknight6]

Hi Cindy, I think there are a lot of good large medical centers that are knowledgeable about mpn's Mayo clinic, Cleveland Clinic, Atlanta, Boston, Colorado, etc. Where do you live?

[Cknight6 in reply to Meatloaf9]

I live in Ventura, California. 🌺

[Meatloaf9 in reply to Cknight6]

I have read that Dr Jason Gotlib at Stanford is an excellent mpn specialist, might be closer to you

[Ccsintx in reply to Cknight6]

I know you posted about going to MD ANDERSON a long time ago but if you haven’t gone - it is very good. And there is a place to stay free of charge called the Ballard House. Hope you went to MDA. I am fortunate enough to live close by

[Meatloaf9]

If you go to mpnforum.com/list-hem./ you will find a list of patient recommended hematologists for mpn treatment and there are many others in california that are listed. Some in LA and some in San Diego which might be closer to you, I don't know if they consider themselves mpn specialists. But patients have recommended them. Good luck in your search.

[Cknight6 in reply to Meatloaf9]

Thank you so much! I will look at this list. This helps me a lot!

[Jerrymohler]

Hi Cknight, I’m 72 and have ET. On ‘Hydrea’ for three years to lower/control platelet count. The drug was lowering platelet counts, but also other blood cells. My hemoglobin dropped below eight with the decline in red cells. I was very ill. Primary care doc said I needed two units of blood (transfusion). But he sent me to my hemotologist/oncologist for the procedure. Oncologist said my body/blood wouldn’t hold a transfusion. The nausea was horrible but was scheduled to have a series of (very expensive) injections that would take 4-6 weeks to make me feel better. I stopped Hydrea immediately. After three I was considering suicide, but after four I began to feel better. Two months later still off Hydrea and CBC. I will probably, very reluctantly resume Hydrea. But only with biweekly CBCs.

All the best to you,

Jerry

[Cknight6 in reply to Jerrymohler]

Hi Jerry. I’m so sorry. It must have been terrible for you. I’m glad to hear you started feeling better though. You will definitely be in my thoughts and prayers.

Cindi

[Kelly2 in reply to Jerrymohler]

I would be scared too! but didn't you have blood tests often to see what happened with the treatment?

[Cknight6 in reply to Kelly2]

Hi Kelly2. Yes, I have blood tests every month.

[hunter5582]

Would definitely get a second opinion from a doc who is a true MPN expert. I found one from this patient-recommended list mpnforum.com/list-hem/ . One thing that the MPN-expert doc I consulted told me is that bone marrow biopsies are often done/read wrong. The skill and knowledge of the docs doing the procedure varies quite widely. This is very important to get right. I did have to travel about 2 hours to consult with the expert-doc - but definitely worth it. From the rest of this thread, it sounds like you would really benefit from seeing a doc who really does know MPNs.

[Cknight6 in reply to hunter5582]

Thank you so much! I am in the process of getting an appointment for a second opinion now. I appreciate everyone’s help so much. This forum continues to give me hope.

Kind regards,

Cindi

[Ccsintx]

I know you posted about going to MD ANDERSON a long time ago but if you haven’t gone - it is very good. And there is a place to stay free of charge called the Ballard House. Hope you went to MDA. I am fortunate enough to live close by.