« An improved understanding of these myeloproliferative neoplasms, including the identification of driver mutations in JAK2, CALR, and MPL, has opened the door to treatments that enable the natural history of these diseases to be altered »
« There are preclinical data suggesting [that interferons suppress] the JAK2 clone, although there’s probably some advantage even in patients who don’t have the JAK2 mutation,” Palmer said »
As always, thanks for the up to date reports.
--
ET
<<Generally, (ET) patients will present with an elevated platelet count, and they get sent to hematology>> In my case I had 1000+ PLT but Dr sent me in for just slightly too high WBC, interesting priorities.
<<Patients with CALR mutations have been observed to have an approximately 50% lower risk of thrombotic events than their counterparts with JAK2 mutations>> but they still suggest aspirin for all MPNs
In contrast that INF may be disease modifying: <<hydroxyurea is not a disease-modifying agent, (but) it helps control blood counts>>
Besremi is in the SURPASS ET trial but it's for only 12 months. INF usually needs much longer to show its stuff as noted even in this report. I hope it will have an extension.
INF in ET seems to have good data already: <<Mesa also said that interferons may eventually replace hydroxyurea as the preferred first-line cytoreductive therapy in patients with high-risk ET>>
<<hydroxyurea produced more histopathologic responses.6>> HU providing BM (marrow) response is news. I've been thru that report (ref 6) and can't find that part. It is intriguing if it's in there. Anyway other reports have shown BMR in INF requires at least 4 years while this went for 12-24 months.
This has been an issue that I think is going away last year or two:
<<Mesa) said oncologists may have had negative experiences with interferons from the days..>>
Huge plug for Besremi <<“Ropegylated interferon at low doses is a dramatically different therapy. (vs PEG) If you haven’t tried it, don’t let that prior experience make you hesitate…. It’s quite helpful,” he said. >> The "low doses" part is important detail not all Drs are on board with.
PLT control: <<patients with difficult-to-control platelet counts tend to respond better when anagrelide is given as a combination therapy vs as a monotherapy>> No mention what to combine.
CALR future <<Longer term, there will maybe even be targeted approaches toward calreticulin.>> This likely means the immune therapies, which are not so far applicable for Jak2.
--
PV
<<“If the EPO level is low and the JAK2 mutation is positive, then you have a very good reason to believe that this is PV,” >> I posted a report recently showing that low EPO is common in ET.
99% of PV is positive for either v617f or Exon 12 jak2.
HU and leuikemia <<many patients have concerns that hydroxyurea will increase their risk of leukemia, despite this never having been “borne out in well-done studies>>
Another plug for Besremi <<Of the available options, Palmer was most excited about ropeginterferon>>
If PEG was previously used for PV and is now being superseded by Ropeg, I wonder why it’s taking so long to approve Ropeg for ET as well? Or maybe it isn’t ‘so long’ - it just seems to be when you’re waiting for these things?
It's in trial for ET, "SURPASS-ET"
pubmed.ncbi.nlm.nih.gov/359...
At least in the US, any Dr can Rx it for ET off label (same way PEG and HU are used) But insurance won't likely cover the $180,000 cost since it's off label.
PEG does sometimes get approved for ET, with Ropeg now avail it starts to look inconsistent, except PEG is cheaper.
Broadly it's bureaucratic stuff.
Thank you. Nice and clearly written.
A good summary article. Thanks for posting
Treatment for PV in France
ET treatment is cruel and illogical
Et treatment options 
ET and Autoimmune disease
