In myeloproliferative neoplasms (MPNs), patients reporting pruritus had more symptoms, greater symptoms severity, and were more likely to experience disease evolution, according to results of a questionnaire-based study published in Journal of the European Academy of Dermatology and Venerology. [The 2nd of those terms indicates the study of sexually transmitted diseases- an odd pairing?]
Researchers from Brest University Hospital in France sourced data for this study from the Observatoire Brestois des Néoplasies Myéloprolifératives (OBENE) database. Patients (N=504) with MPNs were given a questionnaire about symptoms and quality of life prior to consultations between 2015 and 2020. Symptoms and severity were self-reported using the Visual Analogue Scale score.
Participants had a median age of 68.6 years, 56.5% were women, 54.4% had essential thrombocythemia (ET), 37.7% had polycythemia vera (PV), and 7.9% primary myelofibrosis (PMF). Most participants (77.4%) had Janus activated kinase 2 (JAK2)-mutated disease. The most common treatments were hydroxyurea (35.1%), pegylated-interferon treatment (8.9%), and anagrelide (7.1%).
Overall, 49.8% of patients reported pruritus. Study participants with pruritus were older (P =.01), more had PV and fewer had ET or PMF (P =.004), more had JAK2-mutated disease (P <.0001), and fewer abstained from treatment (P =.014) compared with those in the non-pruritus group.
"We clearly showed the importance of identifying patients with pruritus, who are more symptomatic and at the highest risk of phenotypic evolutions."
Participants with pruritus were more likely to report abdominal discomfort (odds ratio [OR], 3.8), perspiration (OR, 3.29), fatigue (OR, 2.68), concentration problems (OR, 2.65), bone pain (OR, 2.31), early satiety (OR, 2.27), and inactivity (OR, 2.06) and all symptoms were reported to be more intense (all P ≤.00007) compared with patients without pruritus.
Participants with pruritus were more likely to experience disease evolution overall (19.5% vs 9.1%; P =.0009) and specifically to myelofibrosis (13.9% vs 6.3%; P =.0049) and accelerated phase or acute myeloid leukemia (7.2% vs 3.2%; P =.046) compared with those without pruritus, respectively. [This may be a new finding: Has anyone else seen this analysis prior to this?]
Among those in the pruritus group, nearly one-half (44.6%) had aquagenic pruritus (AP). The patients with AP were more likely to be men (P =.0015), more had PV and fewer had ET or PMP (P <.0001), more had JAK2-mutated disease (P =.005), and fewer abstained from treatment (P =.001) compared with those in the non-AP group with pruritus.
Participants with AP did not report more symptoms or more intense symptoms than the non-AP group. However, more patients with AP had disease evolution (25.9% vs 14.4%; P =.025), specifically to myelofibrosis (21.4% vs 7.9%; P =.003) compared with the non-AP group, respectively. [This is scary to me, given that my AP has not improved one whit so far despite treatment.]
These findings may have been biased, as patients completed the questionnaires prior to consultation with the clinician.
Researchers conclude, “We clearly showed the importance of identifying patients with pruritus, who are more symptomatic and at the highest risk of phenotypic evolutions. Furthermore, we found differences in patients with AP compared to those with non-AP. […] Therefore, clinicians must go beyond simply determining the presence or absence of pruritus to determine whether patients are experiencing AP.”
Disclosure: Some study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures.
Aquagenic pruritus in essential thrombocythemia is associated with a higher risk of thrombosis
Abstract
Background: Thromboses and phenotypic evolutions (leukemia, myelofibrosis) are the most frequent complications in polycythemia vera (PV) and essential thrombocythemia (ET).
Aquagenic pruritus (AP) is not only PV symptom, but is also present in ET. The presence of pruritus in PV is associated with a lower risk of arterial thrombosis. [I suppose this is some good news- although I still hate my AP...]
Aims: To date, no equivalent study has been done to analyze the impact of AP for ET patients.
Materials & methods: We used the data from our cohort of patients with myeloproliferative neoplasms seen in our institution (OBENE database, NCT02897297). We collect information at diagnosis, presence or not of AP and all types of complications during their follow-up. To avoid masked PV, all JAK2 positive cases were tested isotopic red mass cell if appropriate.
Results: Among 396 ET patients, presence of AP was found in 42 (10.6%). ET patients with AP were more proliferative, more symptomatic at diagnosis and more difficult to treat. Furthermore, they presented increased risk of thromboses (30.9 versus 17%, P = .03; OR = 2.2 [1.01;4.66]) and phenotypic evolutions (33.3 versus 13.3%, P = .0007; OR = 3.2 [1.44;6.77]), during follow-up.
Discussion: Aquagenic pruritus is classically associated to PV. But we confirmed here that AP is also present in ET and characterizes patients with higher risk of morbidity (thrombotic events and phenotypic evolutions).
Conclusions: The systematic determination of the presence of AP in ET patients should permit us to better identify these high-risk patients for better management and follow-up.
thanks for finding this paper, I skimmed it and will read it in detail soon and hopefully report further. In the meantime I am a bit sceptical and put any worrying on hold if you are a itcher.
I may be a plug for Rux by Novartis, although it says only something like 15% on Rux got itch to go away and it wasnt any better than HU for itch, that doesnt sound right, most on Rux will lose itch , its also common knowledge that few on HU will get rid of itch. I have never heard of the writers of this paper and I am not sure its contents adds up, more to follow.
Ah and one other thing , up to now I have read that those who itch live longer, ie contradicting this paper, I also learned over the eyears that not to take too seriously one paper saying something , especially if by unknown authors and sponsered by the drug company. Back in 2018 a paper came out supposedly showing Rux brought out more mutations in very sick MF patients, everyone got excited including me and it was shown later ie nowish in reality Rux reduces Jak2 % and for some patients they can lose Jak2 and also other mutations, in otherwords the opposite. My personal view is if something is written by lots of experts over a period who are well known I take it seriously. Otherwise its a maybe.
Thanks, my comments about concern for my disease process might best be regarded as hyperbole and some measure of frustration with my progress being expressed in a forum where they are acceptable as venting [vs expressing them to SWMBO- who takes them too much to heart and is already all too worried about my health and possible lack of longevity] , , but I think the second study does support your point about the "benefits" of pruritus, as it does document the reduction in arterial thrombosis which is apparent in AP sufferers relative to those w/o AP.
Thanks again!
PA
PS: It may be that they're unfamiliar because we're not used to dermatologists taking any interest in our skin issues.
I know for my personal experiences, that they seemed completely unfamiliar with and unhelpful with my AP issues both before and after my PCV diagnosis was made.
This part points to a possibly confounding correlation
" Study participants with pruritus were older..more had PV and fewer had ET or PMF (P =.004), more had JAK2-mutated disease"
These are also risk factors and could be a cause rather than an effect. One could reverse it, "these risk factors increase odds of AP and progression." This looks more intuitive. But they may have corrected for it in the detailed text.
Since I have ET and AP, I asked my hematologist (an MPN specialist) about this study. I was told that the findings regarding ET and AP indicating increased disease progression were no longer considered valid and that I should not worry.
Glad to hear that, and honestly, both studies suffered from a problem that is very common in all MPN-related studies- a small sample size- which is related to the relative rarity of our diseases.
On the other hand, the first study I posted was really pretty dern recent, being from 7/22, so I'd be interested if you could get your hematologist to provide a reference that I could read in support of his/her advice.
I was asking about this..Now I'm very scared abt the risks of progression. From what I've seen in papers and asking around it's true that asymptomatic polycythemia vera patients might live without evolution for longer periods of time.
Please don't be scared or worried about it, or at least, do so with a healthy dose of skepticism.
Please also see Ainslie's comments above as well.
That said, per my MPN-specialist, my chronically elevated Hct and Hgb counts, which pre-dated my symptoms consistent with Aquagenic Pruritus by a minimum of 5-7 years, and my eventual diagnosis with Polycythemia Vera [JAK2+] by at least 10-15-years based on her review of my online medical records, and would have justified a much earlier diagnosis of PV.
Personally, although it would have been nice to know that there was a known reason for my severe persistent itch and the other less pronounced symptoms, I'm happy that my diagnosis was delayed until there were clearly better alternatives to aspirin and HU.
SWMBO is less sanguine because she [for some unclear reason] wants me around as long as possible, and didn't care for the life expectancy estimates quoted in the reading she did about PV.
I also agree with MAP44: "Focus on something positive and move along, live your life. ❤️"
You are right the article you referenced is fairly recent. I had a consultation with my hematologist recently after I messaged you and she told me that any change in symptoms is some kind of progression (at least in terms of symptoms) but whether that means we are more at risk for disease progression remains to be seen. Before I started taking Peg, I had other symptoms like numbness in my arms and legs and some tingling. Those symptoms have gone way with Peg and my platelet level decreasing, but out of the blue came the AP. So, I have traded one symptom for another. I continue to use Beta Alanine which helps a lot. I can shower without any problem when I take 1/2 tsp in a glass of water at least 1/2 hour before showering. I have another hematologist in NY who is a specialist and I will ask her and get back to you.
Hi PA, I am using Nutricost Beta-Alanine in powdered form. I get it on Amazon and delivered to my home in Northern Virginia. Do you have access to Amazon? I'm not sure its the best but it works for me and my internist, hematologist and dermatologist think it is fine to use. Dermatologists and as well as neurologists are consulted about AP and have been happy to hear about the good results I have been having with it because otherwise they are pretty much at a loses in helping people.
I live in NYS under [just south of] the big lake, so Amazon is not going to be a problem.
While I just hate their search engine, I have nonetheless had a Prime membership since early on, so that's not going to be a problem either.
I'm not looking for "the best", just something that I can have some confidence isn't a form of snake oil, or more likely, an inert powdered substance like talc or lactose, as has been found in more than a few OTC supplements.
I have not had any success in being able to take showers of any kind, as even a sink/'sponge' bath [or honestly even having my shirt off for more than a few minutes] results in a protracted episode of AP [the severity of which I think is not well understood by healthcare professional, because at least for me, it's more like painful pins and needles than just itchiness].
I will be trying it [Beta-Alanine] ASAP- so thanks again!
Hi PA, Please let me know if BA helps you. Remember to wait at least 30. minutes, preferably longer to take a shower after drinking BA (stir 1/2 tsp in a full glass of water for at least 20 seconds before drinking it). I think it helps if I take BA in the morning, exercise and then shower. In other words, once it gets into my system in the morning it seems to be effective most of the day.
I wanted to pass on some good news. As I have told you, BA has made it possible for me to bathe and shower without a problem. I was a swimmer before I started having the problem with AP. I have never tested whether I could swim without a problem using BA (I was afraid I would find out that I would still have a problem). Today I got brave and went to the pool. I was able to swim (around 40 minutes in the pool) without the horrible stinging and burning I previously experienced. I was VERY happy and am hoping that BA helps you as much as it has me;
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