Median survival is ⁓15 years but exceeds 35 years for patients aged ≤40 years. Risk factors for survival include older age, leukocytosis, abnormal karyotype, and the presence of adverse mutations. Twenty-year risk for thrombosis, post-PV MF, or AML are ⁓26%, 16% and 4%, respectively.
Risk Factors for Thrombosis
Two risk categories are considered: high (age >60 years or thrombosis history) and low (absence of both risk factors). Additional predictors for arterial thrombosis include cardiovascular risk factors and for venous thrombosis higher absolute neutrophil count and JAK2V617F allele burden.
Treatment
Current goal of therapy is to prevent thrombosis. Periodic phlebotomy, with a hematocrit target of <45%, combined with once- or twice-daily aspirin (81 mg) therapy, absent contraindications, is the backbone of treatment in all patients, regardless of risk category. Cytoreductive therapy is reserved for high-risk disease with first-line drugs of choice being hydroxyurea and pegylated interferon-α and second-line busulfan and ruxolitinib. In addition, systemic anticoagulation is advised in patients with venous thrombosis history.
Additional Treatment Considerations
At the present time, we do not consider a drug-induced reduction in JAK2V617F allele burden, which is often incomplete and seen not only with peg-IFN but also with ruxolitinib and busulfan, as an indicator of disease-modifying activity, unless accompanied by cytogenetic and independently-verified morphologic remission. Accordingly, we do not use the specific parameter to influence treatment choices. The current review also includes specific treatment strategies in the context of pregnancy, splanchnic vein thrombosis, pruritus, perioperative care, and post-PV MF.
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Manouche
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I just skimmed this paper, it certainly put the cat amongst the Pigeons. If definitely shows these expert docs don’t agree. Tefferi is another one who reckons non of the drugs modify our MPN’s and he seems un impressed by lowering allele burden significance . Likes HU and Bisulfan, seems okayish with Peg but seems to prefer venisect for low risk patients. Doesn’t seem much a fan of Rux for PV . What always amazes me is how different these experts see it, which in reality means some of them are wrong, but the tricky bit is which docs are wrong. I suspect that many experts won’t agree with Tefferi and many/some won’t agree with Dr Silver. I wish they would all get together and hammer it out so us patients have clearer info. Interestingly for such a prominent MPN doc it’s surprising that he hasn’t in my experience over 10 years has not spoken at any of the conferences I have been to and that includes the doc to doc one in NY.
I should probably add so as to avoid time consuming and likely unproductive debate that I don’t think I agree with much of what he has written in this paper.
Tefferi doesn’t seem to be Hasselbalchs’ best friend.
« In this context I am worried by the reported claims by Dr. Ayalew Tefferi that IFN is no better than HU and busulphan in the MPNs. If Tefferi has said so, it is problematic that a colleague, who may be aware of his great impact on the “scientific community ” and MPN-patients’ care, stands up and not tells the truth ».
I think that open letter was some time ago, maybe 2014, if accurate Tefferi views on Peg are a bit negative according to Hans , in Tefferi 2023 he seems a bit more positive about Peg etc but I still think the extreme opposite views on treatments these experts display is concerning. After all if say Silver has actual stats which in his view show life extension or free from progression extension, it’s odd that others such as eg Tefferi and Clair Harrison say in 2023 that none of our drugs slow progression, something off there it seems. Don’t they talk to each other to hammer it out, seems odd to me.
I think this points out the underlying truth about this disease - there is no single right answer for everyone. I have my own experience to draw from, and those of others who post on this forum, and the common thread is this is a bit of a challenge to find what works best for the individual patient. As has been posted numerous times, this underlines the importance of being informed and being an advocate for oneself. Leaving yourself to the whims of any particular doctor could be quite hit or miss. Many thanks for the post.
Thank you for posting this. A few points I will discuss with my MPN specialist at Cleveland Clinic next month. He actually sounds a lot like my Doc in his views. Best to you.
‘risk factors for leukemic transformation included older age, abnormal karyotype, leukocytes ≥15 × 10(9)/l and treatment exposure to pipobroman or P32/chlorambucil, but NOT to HU or busulfan’
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