Hi all, I'm 65 and was diagnosed last summer via bone marrow biopsy and genetic testing as having JAK2 positive ET. I started taking hydroxyurea 3 times a week, and my platelets are now down into the 500s. I had an appointment last week with my hematologist/oncologist and brought to her attention that my RBCs and Hematocrit have been continually rising for a year, with my HCT running between 49 - 55 and RBCs over 6, so she placed an order for a phlebotomy and IV hydration (my WBCs and Hemoglobin are hovering at the high end of the normal range). I asked her if I have PV or should I be tested for it, and she said no, that it wouldn't make any difference in my treatment. She even said I could stop the HU if the phlebotomies were successful, but the order she placed is only for one every 3 months which seems infrequent to me?
The hydroxyurea gives me a terrible headache that I think is caused by dehydration as I find it very difficult to drink as much water as they recommend when taking it. I recently went back to work, so I had to cut the HU back to twice a week because of the headaches, but I haven't stopped it completely yet as my first phlebotomy isn't until Feb. 10th. My questions are...do you think I should push to be tested for PV, and are phlebotomies enough to control MPNs, and if so, how soon do you see results in your bloodwork and how frequently are they usually done? I'd sure appreciate hearing your thoughts and experiences with them, thanks
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Maximo06
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I would definitely seek a clear diagnosis. ET and PV are not treated the same way. Your diagnosis and individual profile do make a difference in your treatment plan. Suggest seeking a second opinion from a MPN Specialist. Here are two lists.
If hydroxyurea is giving you headaches that are interfering with your ability to function, then you may be considered hydroxyurea-intolerant. There are other options to treat both ET and PV. Pegasys is recognized as effective for both. Here in the USA, Besremi and Jakafi are only the two FDA approved medications for PV. (Note HU is in common use off-label for ET/PV).
If you do indeed have PV, then keeping HCT 60 includes cytoreduction. Not all docs follow the age protocol however. My hematologist did not, individualizing the treatment plan based on the patient's profile.
I was HU-intolerant and it did not control the HCT adequately for my PV. Did OK on platelets, but I experienced toxicity even at low doses. I tried phlebotomy-only for a while but the iron deficiency symptoms were worse than the PV symptoms. I have done much better on the interferons. I started on Pegasy then switched to Besremi. The IFNs have been very effective and have had minimal side effects. Much easier to tolerate than either HU or phlebotomies. Note that we are all different in how we respond to the treatment options.
You are asking very reasonable questions and have a legitimate concern. While we can make suggestions, you will need to seek your answers from a MPN Specialist.
I'm not a doctor, but your blood counts suggest a classic case of PV that's getting more aggressive with time. I'd try to get an Erythropoietin (EPO) blood test done. Inexpensive. A low test value of 2-5 helps confirm PV, in lieu of a bone marrow biopsy. Your doc sounds like one of those that only cares about a patient's risk of a blood clot and that's why they only offer HU, Jakafi and phlebotomies to their PV patients.
But some docs consider Pegasys or Besremi interferon to be the best and most ethical treatments for PV, if the patient can afford and tolerate them, because they not only help normalize blood counts and reduce or eliminate the need for phlebotomies, they usually slow or stop disease progression to secondary myelofibrosis and other, more lethal forms of leukemia. As blood counts slowly normalize while taking interferon, PV symptoms tend to diminish as well. For complicated to explain reasons, lots of hematologists/oncologists won't offer interferon to their patients or even mention it. So if a patient faces that obstacle they may need to seek out an interferon friendly and experienced hematologist. Or confront their existing doc with medical journal evidence like this: ncbi.nlm.nih.gov/pmc/articl...
it sounds as if your ET is maybe changing to PV which is fairly common, I am a bit concerned about by our doc, the priority right now is to get your Hct to under 45 if male and ideally 42/43 if female, above those numbers there is a exponential increase in thrombotic risk, don’t want to worry you but safe is better than sorry, you need venisecting until your Hct is down to those numbers and kept at those numbers by venisecting or whatever meds you settle on.
You need a clear diagnosis of which MPN you have and your doc should do that, if it were me I would if poss try to find a better doc or at least see a MPN expert every so often to get proper direction and often the expert can keep your local Haem in the right direction by writing to them or calling.
You say you brought the high Hct to your docs attention, that’s a bit ridiculous as it should have been her flagging it up not you.
Venisection will only control Hct, Hgb, RBC etc not platelets or WBC. WBC near top of range is not an issue and platelets around 500 are fine unless you have symptoms or other health risks, most expert haems are ok with platelets around 1 million unless symptomatic or other health risks.
Your doctor sounds a bit lazy and uninformed. You definitely need to figure out if it’s ET or PV because, as Hunter says, the treatment is different. I hope you can find a mpn specialist in your area.
Strongly agree with all that's been posted here. I've had pv for 3 years, relying on phlebotomies for the first 2 and starting hydroxy last summer. I'm up to 1500mg per day and will probably be trying besremi in the future because hct isn't responding. Your doctor sounds a bit sketchy to me honestly and I'd consider a second opinion at the least. The only thing I can add is to make sure they are monitoring you for iron deficiency and/or anemia. Good luck. Saltmarsh
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