Stubbornly high Hematocrit levels?

[Innessant]

I am Male, 68 years old diagnosed with PV in 2014. Used aspirin, regular phlebotomies and in the last 2 years Hydroxyurea ( 500mg>1000mg) as the HCT levels kept creeping up to mid 50's.

On the positive side I feel fine and in general am in very good health with no apparent side effects of the disease or the cytoreductive medication.No problem with phlebotomies or heart health.

However Dr is concerned that my Hematocrit levels are stubbornly in the low 50's and I am having up to 6 phlebotomies a year. I will get a spleen scan and see after that.(It has grown larger)

Dr appears puzzled at why I still produce so many red blood cells at my (advanced) age.Thinks it may be diet related. I eat a relatively healthy diet ,not overweight and am fit.

Any comments anyone?

[hunter5582]

It sounds like you need to consult with a MPN Specialist who better understands how PV can manifest. Some cases are more treatment resistant and are refractory to hydroxyurea. That is what happened to me. I had to do phlebotomies every three weeks in addition to taking a dose of HU that I could not tolerate (500mg/day). I responded much better to the interferons, Pegasys then switched to Besremi. I am maintaining a complete hematologic response only needing phlebotomy 1X/year to keep HCT < 45%. Moreover, my JAK2 VAF has reduced from 38% to 10%. I also feel better now than before I started in the interferons.

The reason your body makes too many red blood cells is because of the JAK2 mutation. It has nothing to do with age. Unless you are consuming huge amounts of iron it also has nothing to do with a healthy diet. It has to do with the fact that you have PV. This is the nature of the disease. It is also the nature of any MPN that we are not all the same in how we respond to treatment. We need individually tailored treatment plans based on our unique MPN profile.

There are other treatment options that may work much better for you. Besremi or Jakafi are both effective treatment options that may be more effective for you. Suggest that you consult with a MPN Specialist who can best advise you on next steps.

Wishing you success moving forward.

[Innessant in reply to hunter5582]

Many thanks Hunter for sharing your experience and advice.I was not aware that you had such a high frequency of phlebotomies on your earlier HU regime.My JAK2 VAF level was only measured on diagnosis (35%) 10 years ago.I'll let you know how things develop.

[RoundTheWorld]

I was puzzled by your comment ‘Dr appears puzzled at why I still produce so many red blood cells at my (advanced) age’ too as the majority of MPN patients are in the ‘more mature’ bracket.

[Innessant in reply to RoundTheWorld]

He is an MPN specialist. I think I wrote a confusing sentence.He was puzzled why all my other blood readings are well managed and have decreased (WBC,Platelets etc) in line with HU treatment but the HCT seems difficult to lower.He said that if I had serious progression then the WBC counts would be far higher. It was my understanding that HU works on ALL blood cells and Platelets . Thanks for your comment.

[RoundTheWorld in reply to Innessant]

Ah ok - I see. (It’s such a balancing act between the numbers!)

[DougyW]

Are you under a Haemologist? Your age is not that old and and you should not expect that to suppress blood cell production. Do you have any other departments that may have an MPN specialist? As well as looking at Hunters advice you may want to look at moving to a different doctor.

[Innessant in reply to DougyW]

Thanks for your comment. Doc is a haemotologist and MPN specialist.In the past he warned me that phlebotomies may be less tolerated with age. I largely ignored this as I have no problems at all with that and quite like the "simple" physical removal of blood cells thus relieving the spleen of excessive strain.Ill update you all when I learn more.

[Loubprv Volunteer]

I haven't read any other replies but your doctor sounds as though he doesn't know what he's doing. Might I suggest an mpn specialist? Of course your hematocrit will keep going up despite your age. That's the nature of the beast, presuming you have pv.I have pv and am 70.

I m on Ruxolitinib and my haematocrit is well below 40. Please seek the opinion of an mpn specialist, and transfer to him/her

I did the same 12 years ago and now have superb treatment

Suggest you have a look at mpn voice.org.uk presuming you're in the UK?

Best wishes and don't worry, someone will sort you out. My haematocrit was 74 when I was diagnosed in 2009 and I lived to tell the tale. I had a venesection every fortnight and was on HU as well!

Louise

[Innessant in reply to Loubprv]

Thanks Louise for you encouraging message. I appreciate it .He is an MPN specialist and I'll stick with him for now as he is generally okay. I live in Thailand and fear the (current) huge cost of Jakafi which is not an issue on the NHS. I'll switch and pay if I must but hanging on for now as I am fine with HU which is very low cost.My HCT was 67 at its worst ...Thanks again

[Loubprv Volunteer in reply to Innessant]

Thailand! Wow!Completely understand but as Hunter says you could always try besremi All the best

[sbs_patient]

I completely agree with Hunter. I was diagnosed with PV in 2015, first treated with phlebotomy, alone then HU. I had serious allergic reactions to HU, and I was getting monthly phlebotomies. I eventually consulted a MPN specialist who put me on ruxolitinib (Jakafi) about 2.5 years ago. Things have been stable since then.

[Innessant]

Thanks for sharing your story. It is an option but I live in Thailand and Jakafi is extremely expensive which few people realise unless they have to pay. Sticking to HU and phlebotomies for now as I am free of all complications to date . Like all of us we fear transformation of the disease to the next level.

[Luthorville]

A mid 50s HCT is considered quite high. A lot of the biggest issue and concern around PV is that it leads to a large increased risk in stroke. Having an elevated HCT (and platelets) are significant contributors to this risk. This risk is likely elevated further by your age.

I would also suggest talking with an MPN Specialist, and echo all of Hunter's comments. Both Besremi and Jakafi can have an impact on helping to moderate your HCT. At a minimum, though, with your HCT well over 45, I would think that you should absolutely and immediately be getting a phlebotomy -indeed, probably multiple over a few weeks, in order to bring down your HCT to below 45 and reduce your risk of having a stroke.

[Innessant]

Thanks for your advice which is appreciated.