ET Progression?

[Trueblue8]

Just wondered if anyone can offer any advice on what signs and symptoms to look out for that might suggest that ET has progressed to MF.

Prior to my diagnosis of ET JAK 2 early last year, I had several months of left side abdominal pain, I had a scan but nothing untoward was found. I also had severe itching after showering. The abdominal pain has recently returned, although it is only sporadic.

My diagnosis was quite by accident (a blood test for my prostate cancer) and I'm now on low dose aspirin and 1000/500 hydroxycarbamide alternating daily.

I have my bloods checked every 3 months and have had some issues with low haemoglobin and low MCV(not really sure what this is?). I also have recurring itchy rashes on both my upper arms and blotchy skin on my ankles and calfs. Furthermore, the bone pain in my lower limbs, particularly after periods of rest, seems to be getting worse and my level of fatigue has got far worse, I do exercise regularly though.

Any advice would be gratefully received.

Thanks

Ian

[Otterfield]

I have recently been diagnosed as having progressed from ET to MF. Before diagnosis I was increasingly fatigued and breathless, and my abdomen was swollen from an enlarged spleen. If you have any doubts at all, then do ask for a BMB. If you progress, the sooner treatment starts the better. Good luck.

[Trueblue8 in reply to Otterfield]

Hi, thank you for your reply. I will speak to my consultant next time I go to the hospital. I'm not massively impressed with them to be honest, in particular the CNSs, who are quite dismissive of ET.

[Chaz1 in reply to Trueblue8]

Are you seeing a MPN specialist. I'd talk with them aboit your concerns & remember that you can asl for a second opinion. We opted for a second opinion for hubby after initial diagnosis of ET as he was diagnosed, given aspirin & sent away for 6 months despite being very symptomatic. Was obvious from bloods that it wasn't just ET and had referral & diagnosis changed to ET/PV. Although he's symptomatic still he gets a bit of relief with having venesections & his bloods are generally stable. I can't say they are massively attentive & can be dismissive of how he feels, both CNS & consultant but he's being treated better than he was.

[Trueblue8 in reply to Chaz1]

Thanks for your reply. Do you mind me asking what the issue was with the bloods? It has crossed our minds that it might be PV, which I will mention when I'm next seen at the hospital by my consultant, who isn't an MPN specialist! Also not sure when that will be as bloods currently done at my local surgery.

Thanks

Ian

[Chaz1 in reply to Trueblue8]

His HCT, red cell count & WBC counts were all high & obviously increasing from the first couple of tests. As soon as we saw the consultant at the new hospital he said he suspected PV based on the bloods. Was a massive relief to realise that the consultant did know what he was talking about. It's really hard as we've all been raised that Drs are important & know their stuff but it's apparent that you really do need to advocate your care. I'd personally have a look at your past blood results & see what they look like, see if anything is apparently changing beyond the platelets & make an appointment. Is the consultant having telephone appointments with you, these have been working well. If you are concerned you can ring & book an telephone appointment. Or talk to your GP about your concerns.

[Trueblue8 in reply to Chaz1]

Thank you for the information and the good advice, it's much appreciated. Ian

[Chaz1 in reply to Trueblue8]

No problem l. Hope you get some answers soon. Take care

[AndyT]

Hi Ian - I’ve been monitored for my ET for 17 years with no signs of progression.

My consultant has said the main thing they will react to as a possible sign of progression to MF is a consistent reduction across all blood counts, suggesting that the bone marrow is slowing down its production of them.

A BMB can also establish the degree of fibrosis in the bone marrow but I’ve been told it would only be done if blood results indicated signs of possible progression.

[Trueblue8 in reply to AndyT]

Thanks for your reply, it's much appreciated. I'm probably worrying about nothing but will speak to my consultant.

[Cja1956]

I was diagnosed with post ET MF last year. I have been seeing the same hematologist since 2008 for Et, but around 2016, he told me I also had PV. By 2018, I he told me I had become anemic. My platelets were in the 700’s but my hmg and other red blood cell counts were very low. I became extremely weak and fatigued and unable to work a full day. I was getting constant infections and ending up in the emergency room. I asked him for another bone marrow biopsy because I thought I had progressed to MF but he didn’t think so. So I found an MPN specialist in 2019 and she confirmed my suspicions. She has been a godsend. So my advice is if you have doubts about your diagnosis, I agree with the others that you should seek a second opinion. Good luck.

[Trueblue8 in reply to Cja1956]

Thanks for your reply it's very much appreciated. Ian

[hunter5582]

" MCV stands for mean corpuscular volume. There are three main types of corpuscles (blood cells) in your blood–red blood cells, white blood cells, and platelets. An MCV blood test measures the average size of your red blood cells, also known as erythrocytes."

The HU could certainly impact your red blood cells since it decreases all hematopoiesis; however, you do need to know what is going on. You have very legitimate concerns. If the CNSs (or any other providers) are dismissive of your concerns, fire them. Assertive patients receive higher quality care. Passive patients do not. It is up to you to ensure you receive the quality of care you deserve.

Others have already given you really good feedback. The only thing I would add is that you really do need to see a MPN Specialist at this point. Here is a list mpnforum.com/list-hem./ .

All the best. Do let us know how things turn out.

[Trueblue8 in reply to hunter5582]

Thank you for the very comprehensive reply and the list of MPN specialists, it's very much appreciated. Ian

[KentBee]

Hi, I also have severe itching on top of arm when I get hot. My consultant said it was unrelated to JAK2positive ET and taking Hydroxycarbamide and Aspirin. Just seems odd that lots of us seem to have this severe itching causing poor sleep.

[Trueblue8]

Thanks for your reply. It's a known side affect of MPNs, so many consultants out there who don't really understand the condition!