ET (confirmed) and fibrosis grade

[Aneliv9]

Hello to all,

I have my BMB results that confirm a mild form of ET and although my Heamatologist told me that i have no fibrosis, in the paper is written: MF-1. How common is this? I live in europe

Thnk you all

[Cecilie]

Hi,

I was initially diagnosed with ET but after my bone marrow biopsy showed grade 1 fibrosis, it was changed to "MPN Unclassified". Apparently it doesn't fit with either the ET or MF diagnostic criteria. I'm confused by this, as there seems to be some disagreement among doctors regarding the presence of fibrosis in ET patients. I wonder why your doctor told you one thing, and then the results show another thing? I would ask for more details from the haematologist. Also, I wonder what they mean by a mild form of ET?

Kind regards,

Cecilie

[Aneliv9 in reply to Cecilie]

I really dont know. Mf 1 is here in Greece mild reticulin fibrosis. But otherwise consistent with ET

[Aneliv9 in reply to Cecilie]

How many years ago you reclassified us MPN-u?

[Cecilie in reply to Aneliv9]

Two years so far...

[Aneliv9 in reply to Cecilie]

I see.. has anyone told you what is the percentage for progression to myelofibrosis? Also. Do you know what mutation do you have?

[Cecilie in reply to Aneliv9]

No, not sure about the progression rate. Since I have some fibrosis I just assume I am heading in that direction, but I really don't know. I am JAK2 positive, how about you?

[Aneliv9 in reply to Cecilie]

I dont know my mutation but sure not Jak+ . I am so afraid to, that i am heading in the direction of fibrosis . That is what my common sence says.. My heam told me that i have 15% of fibrosis progression in the future, and this is scary since i am only 35 with 2 babies. Mild form of ET means that i have boarderline high platelets and very low risk for thrombosis

[Cecilie in reply to Aneliv9]

I think they are only guessing, really, because each case is different. Hopefully things will change in the future with more options available to us with new treatments and medications. Stay positive!

[Aneliv9 in reply to Cecilie]

I hope so..

[hunter5582 in reply to Aneliv9]

Don't sweat it too much. I was diagnosed with ET in my early 30s. Am 63 now. Never had any serious probs even when I progressed to PV 6 years ago. The secondary symptoms have always been more bothersome. Hope uou jave a similar low end course with your MPN.

[Monkey57 in reply to hunter5582]

Got ET also at your age. Wonder how you got thru for 30 years.

Did u take treatments? Do you have kids as also would the disease pass on?

[hunter5582 in reply to Monkey57]

When I was diagnosed with ET in my early 30s the tx protocol was hydroxyurea and aspirin. After about a year, the hematologist took me off the HU and went with aspirin only as the tx protocol had changed. For the next 25 years, my platelets hovered in the los 500s - mid 600s. I never experienced any thrombosis and other serious symptoms from the ET. About 6 years ago, I had some significant GI inflammation, which triggered major reactive thrombocytosis and platelets went up to close to 1 million. The doc at this time determined I was JAK2+ and put me back on HU for about a year. Went back to aspirin only after the platelets stabilized. About a year ago, post-surgery - another bout of reactive thrombocytosis. Back on HU for another year. It also become evident that my ET had progressed to PV and I started a course of phlebotomy. I finally went to go consult with a MPN-expert doc at Johns Hopkins. He determined that I had actually progressed to PV at least 6 years ago and the hematologist had missed it. I am quite fortunate in that I suffered on ill effect from the missed diagnosis. The only direct PV related issue is mild splenomegaly. JAK2 quantitative analysis determined mutant allele burden =25%. The low mutant allele burden has a lot to do with why my MPN symptoms have always been relatively mild. I have always been more bothered by the secondary JAK2-inflammation related symptoms (insomnia, GERD, osteoarthritis, etc.) The MPN-expert doc determined that HU benefits were not worth the risk in my case, so I d/ced the HU. He also suggested I d/c the aspirin, saying at this point in my life the risk of bleeding was greater than the risk of thrombosis (not a standard recommendation) He said "you do not want to bleed in the brain." Turns out he was prophetic. About a month later, I was diagnosed with a hemorrhagic brain tumor, which is surrounded by edema. A blood thinner would not be a good thing for me at this point even if needed for the PV. So for now - it is phlebotomy only and monitor with labs. Once I do the resection to remove the tumor, we will revisit my PV treatment plan. This is all a somewhat length explanation of why each of us with MPNs have our own unique presentations. Our needs are not always the same. Each of our cases need to be evaluated on an individual basis by a doctor who has expertise in MPNs. Most docs, even hematologists, have very little experience with MPNs. Reliance on the standard tx protocol may work for most, but not for all of us. I hope you find the answers you need for your unique case. FYI - MPS are generally not inheritable; however, there is a rare type of Familial JAK2 that can be inherited. My daughter is JKA2+ too - but is asymptomatic - so she is monitor-only. If you have not found an MPN expert, here is a list of patient-recommended docs mpnforum.com/list-hem/ . All the best to you.

[hunter5582]

By mild-ET am assuming platelet levels not terribly high and you have not experienced and thrombosis. All good signs - I continued on that path for 25 years until my ET progressed to PV. Actually still considered mild PV as few issues with the PV too. Regarding your fibrosis classification - it think it depends on which grading system they used. Here is a link to what I believe is the current European consensus rating system ncbi.nlm.nih.gov/pmc/articl... . Definitely worth reviewing with your doc.

[Aneliv9 in reply to hunter5582]

Thank you very much for the interest. I also saw this Table for fibrosis grades, and i also read other articles for bone marrow morphology . I think that this grade of fibrosis along with my other clinical findings, must be the "presentation" (in the bone marrow), of Calr mutation. I will definetely though ask my Heam.

[hunter5582 in reply to Aneliv9]

The hemo can definately tell you whether JAK2, CALR, or MPL. While VERY rare, you can be both CALR and JAK2 positive. Hope you get answers soon.

[Aneliv9 in reply to hunter5582]

I have not had the mutation test yet

[xxbzd]

你好吗？很高兴见到你我是一名中国人，也是一名MPN.我也很消极 et 同时 mf 1 ，这让我感到懊恼和恐惧。我想问一下医生是怎么解释这个问题的？