To be classified or not with the correct MPN

In my quest to get a correct diagnosis for travel insurance I spoke to one of my doctors and he said that my MPN seems to fall in between a few different areas. After I got diagnosed with having the JAK2 mutation following my surgery for blood clots it was suggested I had either pre-fibrotic Myelofibrosis or Iron deficient polycythemia.

I wondered how others have been diagnosed correctly. From what I've learnt it seems it can be difficult to differentiate between ET, PV and MF even under a microscope at times.

12 Replies

oldestnewest
  • I was diagnosed with E.T. 10 years ago. I had a bone marrow biopsy, a scan on my spleen and many blood tests. This was before the JAK mutation was discovered. I have been tested recently for JAK and am negative. I am positive for CAL.

    My symptoms were extreme tiredness and headaches.

    The consultant I saw initially was fairly positive with his diagnosis as was my brilliant G.P.

  • Hi

    I was originally diagnosed as MF but due to lack of fibrosis this was changed, this was also due to confusion originally when i was Anaemic.

    I am now diagnosed as PV, but my consultant often refers to it as Iron Deficent PV.

    Are you actually anaemic, what is your ferritin and other blood levels?

    Paul

  • Hi Paul, Funnily enough when it was suggested I had iron deficient PV it also said in the letter I wasn't iron deficient. I've never had anemia although my levels have slightly gone below the norm from time to time. They think that was due to the hydroxy doing its stuff, however.

    I believe the treatments tend to be similar for ET, PV and MF anyway. Hydroxycarbamide and aspirin, although I'm on lifelong warfarin for my blood clots.

  • Hi

    It does sound like they arent sure.

    I know my iron deficency was as a result of bleeding varices, which was caused by the additional pressure caused by my spleen. I havent been iron deficent now for some time, and my HGB level toddling along nicely at around 13.5. I am on ruxolitinib so that will be helping.

    Im sure when i took out insurance when there was some doubt i was able to specify just a MPD/N and leave it at that.

    Paul

  • Hi Paul,

    I've got an enlarged spleen and grade 1 varices, although mine has been caused by a blood clot in the portal vein leading to portal hypertension. Was you spleen enlargement cause by the same thing?

    Regards

    Mark

  • Hi

    Ive had scans for clots or blockages and nothing showed up so the assumption its due to my PV, which is common anyway.

    Im not sure what grade my varices are, they are gastric varices. Im having another endoscopy on them next week to check how they are doing.

    I had a transjugular liver biopsy a couple of years back which showed by liver was fine, although i do have elevated liver counts. It also showed the pressure in my portal vein is fine, although they believe the varices will be as a result of past portal hypertension.

    Ive not had a 'bleed' as such from my varices in that i could tell, but they have obviously 'weeped' over the years and this caused my anaemia.

    Dont know how you find it, but the varices seems to cause me the most problems when i try and get travel insurance etc....if i disclose PV it goes up a little bit, as soon as i mention the varices it goes up massively.

    Paul

  • Hi Mark

    I was diagnosed with ET in 2012 following a portal vein thrombosis. this was discovered following a ultrasound and confirmed by CT. in quest to discover what had caused it I had lots of blood tests which picked up raised platelets and confirmed I was now Jak 2 Positive. I was referred to haematology whilst in hospital and treated with anti clotting drugs and put on aspirin. They kept checking bloods and after a little while they said they thought I might have ET. This was confirmed following bone marrow biopsy. They were then pretty positive with the diagnosis but it was a process of ruling out other causes of raised platelets first and the Jak 2 thing seemed to be something that raised possibility of an MPN. Best wishes and good luck with it all. Hope you get your insurance sorted. Liz Cx

  • To answer your actual question, blood tests, scans, bone marrow biopsy and very good doctors. It seems to me that all three MPN's can change one to the other and so regular blood tests are necessary as a constant diagnostic tool. Insurance was quite easy when I had PV but harder when it mutated to MF although StaySure came up trumps for a fee!

    Wish you luck and best wishes.

    Linds

  • Mark

    The following may help...

    nature.com/bcj/journal/v5/n...

    Review

    Citation: Blood Cancer Journal (2015) 5, e337; doi:10.1038/bcj.2015.64

    Published online 14 August 2015

    Rationale for revision and proposed changes of the WHO diagnostic criteria for polycythemia vera, essential thrombocythemia and primary myelofibrosis

    Take care, Bryan & Linda, Barrie Ontario Canada, Linda is 67 yrs - Post ET(2004) - MF (High Risk / Advanced) 2011 Feb, CYT387 2011-2013(failed), Jakavi Nov 2013, Pegasys July 2014, Jakavi 15+15 mg daily + Pegasys 45 mcg weekly + Eprex 40,000 IU weekly (9 weeks, then 6, then 4 due to 20+20 Jakavi, between transfusions so far vs. 3-4 weeks)

  • The hospital are providing a letter mentioning PV because the initial diagnosis highlighted the iron deficient PV so it should help with getting a better insurance quote, although they still said it's still difficult to give a 100% diagnosis for my MPN.

    It seems that my blood clots and ongoing portal hypertension as a result still need to be declared on any insurance so goodness knows what I'll be quoted.

  • Tell me about it. I ended up paying 120.00 for 4 days in greece. If i had been able to just disclose PV it would have been about 30.00

  • Hi Mark, I can't advise this strongly enough, go to someone who is recognised as an expert for MPNs. I was diagnosed just over a year ago with MF ( but not treated - but that's a whole other story about my TRULY awful hospital) . It took a visit to Claire Harrison to discover that I don't have MF at all! Turns out I have MDS!! My original hospital would NEVER have discovered this. It is quite alarming how little expertise some haematologists have in the more complicated diseases such as MPNs and MDS. SEE AN EXPERT.

You may also like...