Science & Medicine

The Burning Planet




Surviving PV:  Is normal longevity possible?

The Weill-Cornell A-team is at it again!  If you or a loved one suffer from PV, click on these links, print out the papers and share with your hematologist.  The difference in some cases could mean an extra decade of symptom free life.

At the Georgia ASH meeting back in 2018, authors Ghaith Abu-Zeinah, Joseph Scandura and Richard T. Silver presented a paper on the efficacy of interferon-alpha as the means for PV patients to improve survival odds.  They  published an expanded revised edition of their work in Leukemia last year.

  • The paper provided evidence that interferon prolongs myelofibrosis-free and overall survival.

Now  in a Lengthy Letter to the Editor published in Leukemia, they describe a deep dive into the data from a cohort of 18 State cancer registries and their own Weill Cornell Medicine database.  They matched both groups by Age, Sex and Race, to create PV-Cornell and PV-Public.

To compare PV survival to the general population, they used US Census Data to determine the life expectancy for people of matching age, sex, race and year of birth.

Their conclusions: Overall survival was significantly higher (26.6 vs 15.8 years) for the PV-Cornell groupThe authors attribute these results to “current standards for attentive management  with aspirin, targeted phlebotomy and cytoreductive therapy at an academic medical center..

And about that normal longevity issue? There was no statistically significant difference in Overall Survival beween the PV-Cornell group and the matched US popluation.”  ergo, a normal lifespan.

You can read the letter here:  Normal life expectancy for polycythemia vera (PV) patients is possible 

MPN Inflammation — Proliferation’s driving force

Inflammation: JAKAFI

It started with an NPR report heard over the morning coffee. Seemed to have nothing to do with MPNs. Long COVID is linked to Alzheimer’s, specifically inflammation crossing the blood brain barrier. A series of trials using a powerful anti-inflammatory JAK2 inhibitor seemed to help. Just as it helped in reducing inflammation in immune disease like RA.

JAK inhibitor in my hemotological mindset spells JAKAFI. The drug came to the MPN world when Incyte, working on a med to address rhematoid arthritis found a fast track path to FDA approval via myelofibrosis, a rare disease.  The rest is history.  Jakafi — unable to stop progression of MPNs, bringing with it serious side effects, triggering new mutations, and by masking symptoms contributing to delay in alternatives like stem cell transplant — has sucked up the vast majority of funds and brainpower devoted to MPN research in the past decade.

Still  alone or in combination with other drugs Jakafi became a billion dollar drug and put MPNs on the hematology map.  Plus it’s our best option for improving quality of life for myelofibrosis patients by reducing inflammation and the subsequent swollen spleen that impacts appetite and normal exercise.

Inflammation: When Interferon  fails
Sometimes you can get too much of a good thing. Type I Interferons, produced by nearly every human cell, are among the first responders to viral infection. At the first sign of infection, interferon signals the production of virus defenses in affected cells, including T cells and Natural Killer cells. Interferon also signals neighboring cells to prepare defenses. The resulting cytokine and chemokine storm starts the body’s robust defense…but can also knock out the body’s own Type I interferon A Rockefeller University team studying 1000 COVID blood samples found >10% conttained interferon-neutralizing antiboldies.

Team Leader, Dr. Jean-Laurent Casanova concluded “”What this means is that at least 10% of critical COVID-19 is an autoimmune attack against the immune system itself. “” (SCIENCE) Curiously, over 94% of these interferon-attacking antibodies attack males only. One suggested medical intervention: Introduction of synthetic interferon to bolster immunity.

How INF puts out the fire. Chronic inflammation is the driving force in clonal evolution, MPN disease progresssion, and symptom burden.  Thus sayeth two of the universe’s leading MPN and interferon experts, Hans C. Hasselbalch and Richard T. SIlver in the European Hematology Association’s- HemaSphere, {2021}

The two specialists cite current research, mathematical modeling, and strong historic evidence to support the conclusion that INF normalizing leukocyte and platelet counts “helps minimize the sustained release of inflammatory cytokines and chemokines and concurrentlly improves immune cell function…” And, as we’ve seen from the Rockefeller study of COVID-19 and interferon, adjusting the antibody response to inflammation can permit Type I interferon to do its work without suffering an auto-immune attack.

Inflammation– and your kitchen spice rack.

Jakafi, a potent antiinflammatory JAK2 inhibitor, might have some stiff competition from a humble source. My traditional rice bowl starts by dumping turmeric (curcumin) and ginger in the boiling water before adding salt and rice.  The COVID-19 pandemic added scientific support to my grandmother’s recipe.

Cytokine storm or exaggerated immune response (see item on inflammation and interferon, above) is a feature of both COVID-19 disease and some MPN responses.  Studies of alzheimer effects associated with Long Covid led to the discovery that inflammatory molecules in infected blood may be triggering the onset of Alzheimers.

Clinical trialls of common kitchen spices — including  ginger turmeric, ginseng, sesame seed and coriander — resulted in significant reduction of inflammation in mouse models (Life Sciences, Nov  2021, Ajaikumar et al;.)

MPN Research Foundation–breaking out of the box.

Thanks in no small part to the Foundation, true molecular medicine has been added to MPN therapy, along with identification and characterizatioj of dozens sofmutations — including the  CALR driving mutation — contributin to MPN.  The Genomic magic of DNA manipulation is now mainstream and already applied to some hematologic pathologies.

But despite billions  (with a B) invested in research, clinical trial and marketing, the MPN pharmacy still relies on a few basic meds — two off-label drugs, hydroxyurea, interferon, plus anagrelide, Jakafi and its lookalike, fedratinib, plus Besremi ()for PV patients) … along with a cabinet full of meds to deal with specific side-effects.

In its search for an actual cure, the MPN Research Foundation is reaching out to new disciplines, new seemingly unrelated areas of research. In its THRIVE . initiative, the Foundations is  crossing its normal borders.  And, in the words of MPNRF chief Kapila Viges,, if you’re “interested in research to advance better outcomes and new treatments for MPN patients” you’re invited to apply for a grant.

“The 2022 Thrive Initiative, supported in part by the Leukemia Lymphoma Society, seeks to reach beyond current MPN researchers, to foster collaborative projects from both new and seasoned researchers in other fields ” The 2022 Request for Applications opened on June 15, 2022 with a submission deadline of August 15, 2022.

The Thrive Initiative gives the MPN patient-scientist, another chance to help move along a cure.

Collaborating with MPN specialists or on their own, MPN patients, have played a strong role in initiating and supporting basic research for more than a decade. Starting with Joyce Niblack’s fatigue burden work with Ruben Mesa’s team., MPN patients have been front and center in moving along basic MPN research.

MPN patients have presented posters at ASH and have been lead or participating authors in more than a dozen scientific peer-reviewed papers. The Fatigue Project,  identification of MPN care resources, symposia on stem cell transplant and CRISPR genomics are among the areas driven by MPN patients….

So patient or new, or seasoned researcher looking for a multidisciplinary opportunity to test your MPN breakthrough ideas click on the link and have a look:  This research opportunity includes four funding mechanisms, each with distinct eligibility criteria.

Can Atlanta’s MPN community survive without Bonnie Evans?

Kathryn Hajek Zuehlke: is taking over the leadership of the MPN Greater Atlanta Support Group, Bonnie Evans old turf. Kathryn is planning the group’s first in-person gathering since 2019.
WHAT: MPN Greater Atlanta Support Group Picnic
WHO: MPN patients, families, and significant others
WHEN: 4 PM on Saturday, July 23, 2022
WHERE:  Mason Mill Park, 1450 McConnell Dr., Decatur, GA 30033.
Y’alll come……if you can.

FOOD: notes from Kathryn: “Roland and I will fire up the charcoal grills and provide burgers, hotdogs, and veggie burgers (and buns) as well as plenty of still and carbonated water and paper-goods.  Please bring a dish to share. ”

MPN scientific research — from bit player to superstar
Nine years ago an issue  of TSR described the anonymity of MPNs in the Greater Hematology World. Ignored, more or less despised and shunted off to a corner of scientific and professional meetings, Poster displays and Exhibit Halls of meetings,  Deprived of talent money and motivation.

All that changed when Incyte, searching to advance its new antiinflammatory RA drug found this rare disease — myelofibrosis — that may have offered a shortcut for approval.  In possible need of an antiiflammatory drug. Fast track status  was available to a drug company that wanted to trial a drug. Ruxolitinib was a candidate and the rest is MPN history.

Jalkaf the billion dollar MPN drug piling up profits and vast pools of cash for research possiblities turned the ignored MPN into a star attraction for researchers. The TSR article in that issue — “Gut flora “– outlines our degraded status back in 2013 and kicks off with a lead article of the race by Big Pharma to catch up .  If iou have some on your hands or you’re feeling nostalgic, it’s worth a read.

To access earlier issues or MPNforum services go to the CATALOG

© MPNforum, LLC 2022,, TSR The Senyak Report and the MPNforum Quarterly Journal are licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License. Reproduction is permitted provided full and clear credit is given to authors and MPNf

Comments on: "The Burning Planet" (3)

  1. from spain
    muchas gracias

  2. Avraham said:

    Hi Zhen,
    Thanks for the update on PV and the other interesting articles

  3. Jane Frantz said:

    Thank you again for another informative issue!

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