International MPN News, Science & Opinion

Myelofibrosis with a Silver Lining

Take two aspirin, call me in the morning,

By Zhenya Senyak

You didn’t go to the big New York MPN Symposium.

Why would you?

If I didn’t have a daughter with a spare futon living on the Upper West Side and wasn’t given a press pass there’s no chance I would put my animals, my credit card and myself through the hassle to hear people say things I could better understand in print or on YouTube.

So when I ended up a week later back in North Carolina, I was surprised at how much fun it was, how many new perspectives I picked up. It’s a little bit like a rock concert. You can buy the CD but you miss the mosh pit and the electricity.

Here’s a report on the experience. Soon, enough you’ll be able to see the webcast on the CR&T and MPDfoundation websites, so I’ll try to give you some highlights from the narrow perspective of a an early MF patient who has declined treatment and is now trying to understand an optimum way forward.

When the story opens I’m on my way to see the doctor who first diagnosed my MF, the same doctor who is essentially hosting the Symposium that will take place two days later at a private club on 54th Street just off Fifth Avenue.

___________________________

There is a kind of exquisite agony in riding the M-86 bus entering Central Park in the declining days of Autumn, waiting for traffic to clear so the bus can lurch another five or ten yards, cutting off buzzing yellow cabs and horn-blaring SUVs and BMWs with Jersey plates.

Through the window, pedestrians can be seen, freely charging through stopped traffic, talking and gesticulating to an invisible other in front of them, looking like a lunatic mob until the telltale ear buds or discreet antenna announces a different kind of insanity, the private cellphone conversation in full public view.

The contrast beween the fading Fall colors of the morningCentral Park forest, bordered in rough cut granite stone, and the tension within the stalled NYC bus is very great. We will never make it through the blocked intersection before the light turns red.

At the other end of the route, at the impossibly distant York Avenue stop on the East River, lie the towers of an American elite medical landmark, the spreading village of professional offices surrounding the stone and glass towers housing the glittering medical technology and labcoat wisdom of Weill Cornell, Columbia University, Sloan Kettering and New York Presbyterian Hospital.

Meanwhile, like a thick embolism, there’s a blockage in the road as cars feed into the single artery crossing Central Park. Ahead, Fifth Avenue where southbound traffic once headed for midtown and the Village is now securely parked in the intersection. Gridlock. Somehow, against all odds, the bus driver negotiates a path and with hissing brakes and a series of jolting, jarring surges gets us across on the journey to the East Side.

In one of the high towers on York Avenue sits the Sage of Hematology, the undisputed elder who bi-annually assembles medical high priests from distant places to forge common battle plans. From this place, for two generations, he has taught, healed, researched and published books and papers that have become the basis for a standard of practice. It would be hard to over-estimate the contributions and eminence of Richard T. Silver.

And almost impossible to imagine arriving late for our morning audience. For me, there are also other reasons to worry about this meeting.

It is the eve of another gathering, the 6th Annual Symposium on Myeloproliferative Diseases and I am about to end a two year journey that started from this place. If this damned bus ever makes it through.

It started like this:

Flashback: The Diagnosis


He is saying something profound, I know, this magical doctor in his little examination room on the third floor of New York Presbyterian. He’s small and balding, his voice is quiet, his manner friendly, familiar, his hands sure. He seems like a congenial wizard, filled with knowledge and secrets.

Soon my future will be spread out on glass slides before him to be read in the tea leaves of my stained bone marrow. I should focus, I know, but he has already delivered the punch line. Richard Silver has confirmed I have, myelofibrosis with myeloid metaplasia, and is now speculating I may never have had ET after all, but simply MF from the beginning. Why do I not find this line of thinking interesting?

I’m considering the bone marrow biopsy ahead, recovering from thelong bus ride and crosstown walk to get to this place, tasting the fact that the MF I simply suspected, based on graphs I produced of my CBC history over the past decade, is now a fact walking around the room on two solid, if fibrotic, legs.

The Silver touch

Why I wonder, with these numbers, do I not have an enlarged spleen. But I do. And Silver finds it, easily, and shows me how to breathe and where it is and how it feels when it is only 2 cm below the costal. For all his brilliance and international reputation, his manner is simple and friendly, like a kindly uncle I never had, and business-like without the slightest trace of rush.

It will be a while before he gets me a definitive report based on multiple blood tests (that turned my right arm black and blue) and the analysis of the bone marrow biopsy. Meantime he left me with a recommendation for a hematologist at Duke. He also gave me a copy of his letter to Leukemia summarizing some favorable results using recombinant interferon in the treatment of primary myelofibrosis. He recommends I start on pegylated interferon at a starting dose of 45 micrograms per week.

Rebellion…of sorts

I did not follow Dr. Silver’s prescription to start PegIntron. And after a lengthy multi-day workup at the Duke hematology clinic, and consultation with my hematologist who discussed the fine line between ET and MF I declined to take Hydroxyurea as well.

More than reject medical advice, I rebelled against this early introduction of toxic chemicals and wrote widely within the MPD community of my decision.

My sole medication is a single aspirin daily. Not much of a defense against advancing MF but armor enough, I believed, against thrombosis or stroke. I continued regular CBCs with my primary physician, monitored counts, but saw no hematologist.

Now, two years later, I was about to see The hematologist.

Finally, the bus pulled over to the curb a block away from Weill Cornell, five minutes early for my scheduled meeting with Dr. Silver.

He was warm and friendly and barely recalled me as a patient among the thousands he had seen or helped in the meantime and knew nothing of my rejection of his prescription.

We talked of his long and varied career, he showed me his photographs from the Brazilian jungles and Antarctic, his book Blood Morphology – published with a preface by the original definer of MPDs, William Damishek. And he talked about his role as principal investigator on Anagrelide, his continuing strong support of Interferon and his satisfaction with the turn-out for this Symposium. It was like a backstage meeting with a rock star before a performance to a packed house.

The Big Show

The big MPN NYC Symposium put on jointly by the MPN Research Foundation and the Cancer Research & Treatment Fund on November 2 was a show.

Awash in glittering credentials, dueling therapeutic options, descriptions of (yet more) promising clinical trials, and the quiet clink of heavy duty corporate sponsors, the Symposium was, in a sense, the opening act for another event, the international scientific congress that would take place the following two days at the foot of the Brooklyn Bridge. For most of us, it was the only event.

In the audience, 226 registrants sat at cloth-covered round tables facing a podium and twin screens on either side of the stage in the University Club conference room. Slides flashed, eminent hematologists, professors and clinical investigators spoke, findings were recited, points illustrated, all punctuated by applause. It was pretty jolly as people matched up faces and name tags with people they had only known through MPD lists, Facebook or MPNforum Magazine.

Getting a chance to actually hug Joe and Bonnie after long months following their ordeal – and love story https://mpnforum.com/2011/08/14/a-love-story/ – was worth the price of admission, the airport security checks, the crowded subways and that nervous breakdown of a bus ride.

Joe, Dr. V. and Bonnie

The Big “D” …. A mild surprise to those of us who finally abandoned MPD and embraced the MPN banner is the the tenacious hold the term MPD held on the assembly. From the brochure itself promising a Symposium on Myeloproliferative Diseases” – to Dr. Jerry Spivak’s overt rejection of these diseases as neoplasms in any sense — the term MPD was gaily bandied about by speakers and patients alike in defiance of the World Health Organization nomenclature.

A One-Day Master Class

Dr. Richard Silver presided over a raft of presentations that offered few surprises for those in the audience who followed the chief MPN developments over the past few years.

The organization of the day, however, was brilliant – opening with an overview of thrombosis, moving into a presentation of the genetic basis of MPNs, and following with treatment options, and alternative views of the natural history of these diseases. It concluded with Q&A to a panel of doctors and breakout sessions for each major MPN.

The structure gave the Symposium the hard edge of a one day master seminar.

Mostly, the formal presentations were a refresher course on the JAK2 inhibitors, the untapped power of interferon, the genetic roots of MPNs, and a series of mini-reports on therapeutic options, projects underway at various foundations (MPN Research Foundation, CR&T, and the NCI’s big Myeloproliferative Disorders Research Consortium) with a few side trips to new therapeutic targets like the bone-blood stem cell niche.

Ruxo and the elephant in the room

A main attraction of the symposium was Ruxolitinib. The only JAK2 inhibitor to have weathered the Phase III clinical trial, Ruxo is widely expected to be approved by the FDA next month. Dr. Srdan Verstovsek and Dr, Ruben Mesa presented findings that differed little from those presented at the ASCO meeting five months ago https://mpnforum.com/2011/10/12/ruxolitini/ , But they are like rock stars on tour and you don’t fault the Rolling Stones for doing Satisfaction, again,

There was an elephant in the room, hard to ignore. Only a few weeks

Dr.Ayalew Tefferi

earlier Dr. Ayalew Tefferi’s (Mayo, Rochester) letter was published in the New England Journal of Medicine raising serious questions about the efficacy of Ruxolitinib. Tefferi reported that of the 51 Mayo patients who participated in the Phase I/II Ruxolitinib clinical trials 47 had discontinued due to disease progression, loss of response, or toxicity. Tefferi further reported “serious adverse events” including hospitalization following discontinuation, Additionally, 18 patients (35%) died and 5 patients (10%) had leukemic transformations. He also raised the issue of thrombocytopenia (platelet destruction) and worsening anemia.

While no mention of the Tefferi letter was made publicly, Verstovsek focused on the results of the large scale Phase III controlled trial, that followed the dosage and toxicitiy trials cited by Tefferi. Both the Comfort I trial in theUSand the Comfort II trial inEuropehad similar results in reduction of splenomegaly and improvement of quality of life. He also addressed the need to reduce Ruxolitinib dosage gradually in cases of discontinuation for any reason

(For an interview with both Verstovsek and Mesa with slides showing effects of Ruxolitinnib in its Phase III trial: https://mpnforum.com/ask-the-doctors/ )

An old workhorse spotlighted….Hydroxyurea

A surprise star of the proceedings wasn’t a shiny new designer drug but the old workhorse, hydroxyurea, hydrea Here Dr.Tiziano

Dr. Tiziano Barbui

Dr. Jerry Spivak

Barbui and Dr. Jerry Spivak took strongly opposing positions on the innocuousness of Hydrea. At issue was the possibility of HU being leukemogenic over time, with conflicting papers and studies cited on both sides. The results seemed to be a draw, since Spivak, conceded, when appropriate and the literature supported such use, he would prescribe hydroxyurea. And Barbui agreed that, when combined with other drugs, the risk of leukemia is indeed increased. He would recommend hydroxyurea as a front line therapy for those patients who needed to be treated.

Ron Hoffman raising the question of standard of care for PV patients who require repressive therapy, compared Pegasys vs. HU, the subject of his new NCI clinical study. “Neither is innocuous, both have important side effects. The question is how does that translate to you.”

The real action began after lunch –

The buffet lunch – the lines for which, resembling the long winding corrals of a TSA security queue at LaGuardia, extended along the walls of both sides of the conference room – may have been the chief disappointment of the Symposium, Those fortunate enough to scarf up the offerings were rewarded with basic school lunch fare – pasta, sandwiches, chips, some greenery and a cookie. At least that’s what it looked like as successful buffet negotiators passed by with their food on display. By the time the last of us bellied up to the table all that was left were crumbs and scraps. But why complain? All New York City and its Sabrett Hot Dog and Soft Pretzel carts lay just outside the door,

The event kicked into interactive gear after lunch with the reading of questions from the audience followed by breakout sessions organized by MPN phenotype.

Here’s a sampling:

. “What is the proper aspirin dosage to reduce the risk of thrombosis or stroke?

Dr/ Babette Wechsler: “While it’s true there is no universal dose for anything, in the vast majority of cases a single low dose — ¼ of adult size — aspirin will inhibit platelet function as much as aspirin can inhibit platelet function.”

“Why are there no African Americans, Latinos or Asians at the conference?

Ruben Mesa: In his practice at Mayo Scottsdale he has Latino patients but admittedly the epidemiology of MPNs is not adequate. We don’t know the ethnic distribution of MPN patients

Ron Hoffman: First, there is an admission fee and then you need access just to learn about the meeting from the Internet. The CDC has concluded the data just isn’t there, the epidemiology “is a mess.” In his hospital, bordering onHarlem and Spanish Harlem, “there is a reasonable distribution of MPD patients, Spanish speaking, Asians, African-Americans… medically underserved populations.”

“Do you recommend a regular bone marrow biopsy?””

Tiziani Barbui: Last year we presented a paper. More than 1000 patients with ET had a bone marrow biopsy at diagnosis or shortly afterward without any treatment. We asked pathologists inItaly and at Mayo to review this bone marrow. ..hTey were able to recognize two groups of patients, 15% with early myelofibrosis and 85% with true ET. The two groups differed in terms of survival. ..It was very clear that the evolution toward MF and leukemia was higher in those classified as early MF. It is useful for treatment to know what is happening with the bone marrow at the beginning of these diseases.”

Jerry Spivak: “If I have a patient with the JAK2 mutation I’m not interested in doing a bone marrow for a couple of reasons. It’s expensive, it’s painful…and the patient always thinks I must have leukemia….A man with a high platelet count and JAK2 negative might be a different story. I do it on a patient basis.”

Ruben Mesa: ” The bone marrow gives us a unique insight into the illness, both into the type of diagnosis…is it going to behave like ET or early MF. Secondly the bone marrow can give us a window into the changes in chromosomes, changes in blast percentages, changes in fibrosis. So I do them at some periodic frequency. If we see things changing quite a bit, then we do it with greater frequency,”

Ron Hoffman: “It’s no picnic but less painful than a visit to the dentist. I think there are two standardized systems to diagnose ET, the World Health Organization and the British Committee on Standardization of Hematology and both require bone marrow. I see very little need to repeat a BMB in a periodic way…If you see changes, yes, but It is not our routine practice to sample every year or several years…because it hurts and I don’t think we’re getting significant information. We can do cytogenetics on peripheral blood. ”

Richard Silver: ” I tend to agree with Dr. Barbui. A BMB is mandatory in a diagnosis. It establishes a reticulin and collagen level and becomes a marker. We believe it is important to do a baseline. We find development of fibrosis can be capricious but I don’t think you have to do it on an annual basis. Jerry you seem to be in the minority..”

Jerry Spivak: “I recall that in the Polycythemia Vera Study Group 265 patients early on had fibrosis and it made not a bit of difference. People come to me waving their bone marrow slides thinking they’ve had the disease long enough to develop fibrosis. And I say, I don’t care. If your disease is unchanged nothing has happened. It means the fibrosis, if it’s there, isn’t bothering you…As a physician, I’m going to pick my times [when it is appropriate.]”

“Do you consider MF cancer?”

Jerry Spivak: “Of course not, it’s a reactive process, ultimately a stem cell failure that creates unstable genomic features. Fibrosis is a reaction to that. Treating the fibrosis won’t change that.””

Ruben Mesa: “Myelofibrosis, as Jerry says, is a secondary phenomenon but let us make no mistake. It is a chronic leukemia and it is a type of cancer. I wish that wasn’t the case and it’s why we labor so hard against this illness. How serious a cancer it is depends on the individual. For some people it could be just as serious and life threatening as other advanced cancers but for others it can be more indolent. Giving it that label, although accurate, doesn’t necessarily make your disease worse but it is clearly the correct way to think of the illness.”

Srdan Verstovsek: “I agree. There is no more to say. It is cancer, a malignancy and it shortens life expectancy in people and should be treated as such.

Ron Hoffman “There are a lot of different causes of fibrosis, the symptom we’re talking about in myelofibrosis is a hematological malignancy. There’s good data to support that in patients and in the laboratory. As manyof you unfortunately know, it is a serious illness. If you have an advanced stage of this disease you have very limited survival. So it is what it is.”

 

Robert Rosen, Barbara Van Husen, the MPN Research Foundation

Before the Breakout Sessions

The working part of the Symposium ended with the breakout sessions, 75 minutes face to face with presenters, divided by disease types.

Before leaving the main conference room the co-sponsors David Boule for CR&T and Robert Rosen, MPN Research Foundation, talked briefly. (I missed David’s talk but at the end he announced the dedication of a new Richard T. Silver MPD Center as part of the Weill Cornell Medical Center.)

Bob Rosen, an MPN patient himself, is soft spoken. He presented the mission and history of the Foundation in a straightforward recitation of facts. As his list goes on for a few minutes, the full dramatic impact builds. In its 11 years, the Foundation has not only funded basic research to the tune of $8.5 million – it has been instrumental in creating research groups, tissue banks, genomic sequencing projects, environmental studies, and patient support systems.

One fact from Rosen’s presentation: A Foundation-funded Yale epidemiology study concluded there are 175,000 MPN patients in the U.S.

Dr. Ruben Mesa, Dr. Srdan Verstovsek

The break-out sessions followed a presentation and Q&A format. The only one I attended was the MF session chaired by Mesa and Verstovsek. (Barbui chaired the ET session, Spivak, PV.) The sense of déjà vue was strong since most of the slides and much of the presentation material had been covered earlier https://mpnforum.com/2011/10/12/ruxolitini/ and in Verstovsek’s morning presentation on JAK2 inhibitors and the JAK-STAT pathway.https://mpnforum.com/jak-stat-pathway-follow-the-yellow-brick-road/ But it’s ore than likely my flagging attention was caused by a combination of information overload, working way past naptime… and the slow digestion of a street vendor soft pretzel.

A question from a stem cell transplant patient who has been suffering from Graft Versus Host Disease questioning the on-going complications she experienced after having been cured snapped me out of it. The ambiguous nature of an MF “cure” through SCT was addressed with great subtlety by Ruben Mesa, particularly along with the on-going risk of GVHD. It’s an issue we need to look at. (Next month Marty Prager will take us through his stem cell transplant experience.)

After the show is over…

The return home to North Carolina was a 14 hour slog with a canceled flight, rerouting through Atlanta, providing ample time to reflect on the high stakes craps game we’re all playing. Now, that I have moved to the high risk category by virtue of age, an undeniable risk factor for thrombosis, what therapeutic conclusions could I take away from the day?

(1) Don’t medicate too early, that seems a consensus. Don’t wait too long is another consensus. The definition of too early and too late is weighed on the balance of discomfort and possible impetus to early leukemia on the one hand and premature death on the other. The image of Karl https://mpnforum.com/2011/07/07/remembering-karl-postjune/ is always before me.

(2) The JAK2 inhibitors are on to something, at least the possibility of holding the effects of MF at bay for a time. It doesn’t matter whether you are positive or negative for the JAK2 V617F mutation. These inhibitors work on the JAK-STAT pathway and while there are multiple JAK signaling enzymes the JAK2 enzyme is specifically involved in hematopoeisis. For a brief exhibit on the JAK-STAT pathway: https://mpnforum.com/jak-stat-pathway-follow-the-yellow-brick-road/ I’m JAK2 negative with minimal splenomegaly and stable counts. Way too early for JAK2 inhibitors.

(3) The interferons hold the promise of reversing fibrosis, affecting the marrow and the near certainty of providing miserable side effects until the appropriate dosage is arrived at. Silver pointed out that our suffering from viral infections like the flu are due not to the virus but the load of interferon, a naturally occurring protein – a cytokine – that is active in immune response. The interferon sweepstakes odds increase in your favor if you start early says Silver and become astronomically high against you if you wait too long. And then there are all the novel, highly experimental therapies now in the labs or early trials, TET2, MPLs, Exon !2. blood-bone stem cell niche targets…the list expands.

An option? For early MF, Interferon is still experimental and the others are not even available at the experimental level outside of clinical trial. Like Jerry Spivak said, we all have a right to our opinions, but no right to our facts. And the facts are just not there yet. Maybe Ron Hoffman’s MPD Research Consortium Pegasys vs HU trials will provide those facts. For now, it’s not worth the risk.

(4) Hydroxyurea is perhaps the most widely used cytotoxic substance prescribed for MPNs. It has generally acceptable side effects, will lower counts, but will not effect the progress of the disease and can compound the difficulty of treating progression to acute MF or leukemia. With the voice of Spivak ringing in my ears it’s easy to decline its use.

That, in 4000 words, is my version of The Symposium. There will be a more objective version via webcast at www.mpnfoundation.org and www.crt.org in a week or so. As to my personal conclusions, as before, I have no better strategy than an aspirin a day and watchful waiting. Difference is, now iI feel more confident it’s a path rooted in the best our MPN world can offer…so far.

Comments on: "NYC goes MPD for a day — Advance Subscriber Post" (11)

  1. Carol Johnson said:

    Thank you Zhen, the information is helpful, I can almost taste a pretzel, hear the traffic and feel the unique “vib” that is NYC. You must do what is right for you about treatment – or not, I vote for you to be careful you are are an important part of our extended family.
    Love and Prayers

  2. I’ve seen plenty of patients evolve medically online. I stand by Dr. Silvers’
    advice to you to start IFN. It is easy once you get used to the
    initial side effects and they go away or diminish after about a month
    or so as your body develops tachyphylaxis to the drug. I take a hefty
    6 MU daily and hardly ever feel I need to take an Advil or Tylenol.
    IFN is a biological response modifier, not chemo, many patients lose
    that distinction.

    The take away message, closely watch your spleen, it is a harbinger.
    It is amazing how we adapt to fatigue and counts, so you can’t trust
    yourself to be aware  of when you start slipping. I’ve seen it over
    and over again with a multitude of patients these last 17 years. Get
    periodic peripheral blood smears to look for nucleated RBCs,
    teardrops, etc., anything that shows your marrow is pushing out cells
    too fast, abnormally shaped. It is a good early warning procedure and
    minimally invasive, it just requires a blood draw. A CBC is not enough
    information.

     The future will be with 23andme type companies doing geneome research
    to understand the diseases, and to tailor treatments and to ID
    patients early on.

    Consider IFN to be a miracle drug for some of us that is available in
    the here and now. If it was newly available as a discovery, and then
    in limited clinical trial, psychologically, most MPN patients would be
    clamoring for it.I’ve seen plenty of patients evolve medically online. I stand by my
    advice to you to start IFN. It is easy once you get used to the
    initial side effects and they go away or diminish after about a month
    or so as your body develops tachyphylaxis to the drug. I take a hefty
    6 MU daily and hardly ever feel I need to take an Advil or Tylenol.
    IFN is a biological response modifier, not chemo, many patients lose
    that distinction.

    The take away message, closely watch your spleen, it is a harbinger.
    It is amazing how we adapt to fatigue and counts, so you can’t trust
    yourself to be aware  of when you start slipping. I’ve seen it over
    and over again with a multitude of patients these last 17 years. Get
    periodic peripheral blood smears to look for nucleated RBCs,
    teardrops, etc., anything that shows your marrow is pushing out cells
    too fast, abnormally shaped. It is a good early warning procedure and
    minimally invasive, it just requires a blood draw. A CBC is not enough
    information.

    No breakthroughs in our lifetime, IMHO. One has to realize that all
    the JAK2 research out there, it is being driven by pharmaceutical
    company stock speculators coupled with large medical institutions
    doing the trials, who for better or worse, are so tied into clinical
    trials being embedded into their MO that financially they couldn’t
    stop or curtail if they wanted to. Most of these new compounds are off
    the shelf, thereby reducing developmental costs. That is my honest
    assessment of the current state of affairs. It is pathetic, 9 out of
    10 drugs in trial never make it to market, yet you see late stage
    patients who lay inordinate hope for a cure. The patient symposiums
    are finely crafted recruitment tools.

    The future will be with 23andme type companies doing geneome research
    to understand the diseases, and to tailor treatments and to ID
    patients early on.

    Consider IFN to be a miracle drug for some of us that is available in
    the here and now. If it was newly available as a discovery, and then
    in limited clinical trial, psychologically, most MPN patients would be
    clamoring for it.

  3. thank you for writting about this event.I don’t understand how the doctors debate the “cancer” label…..does it really make a difference? And if you think it does….why and how?

  4. Dear Zhen, your report/review of the 6th NY MPN Symposium brought back many memories of my trip to New York to attend the 5th Symposium in 2009. The characters, their differing opinions, the raft of information and the ultimate feelings of continued confusion about diagnosis and treatment. Like you, amongst my happiest memories I’d count meeting Bonnie and Joe, the view from the venue windows of Central Park in the Fall and the walks I took there to settle a mind which seemed to be in frenetic overdrive.

    This year, a trip to NY was out of the question. Utter frustration at having my proverbial wings clipped due to PE and lung collapse. So I’ve settled for the London symposium on November 19. The view from the venue windows will be Russell Square, Bloomsbury, not quite as majestic and stunning as Central Park, but it does form part of my old stomping ground in the 80s and 90s and it does have a unique old world charm. It follows that, in the light of opinion received since April, of personal interest will be Prof. Barbui’s paper entitled ‘Why do MPDs cause blood clots?’ No doubt my mind will once again lurch into a frenzied state and there’ll be no chance of dozing during the long train journey back to Devon. Many thanks Zhen for your great summary. I hope you are now fully recovered from the Big Apple experience!

  5. Cyndy Morreale said:

    Thank you Zhen for a great explanation of the symposium and the different players. I found the bone marrow discussion most interesting. Sam’s last marrow test showed nothing conclusive when the Dr was certain Sam might have progressed to Leukemia. 5% blasts in the periphreal blood, but I think that was the same awhile back .. They said his marrow is either so fibrotic they can’t get a reading or so full of leukemia and can’t get a reading or ??? We’re back in the dark … I wish I had a crystal ball these days . He’s back in the hospital again after complications from last weeks falling on glass episode.

  6. Zhen my friend, I am away from home but I took the time to read this (I am risking my life doing this, my son is getting married this weekend!). You did a very good job reporting on the conference (Ok, I miss Dr. Tefferi saying “show me the facts”) but let me tell you one thing, I really do not like the fact that you do nothing to fight your MPN, for me it sounds like the shoemaker is walking around barefoot. One last thing, DON’T PANIC!

  7. Fabulous article, thanks for taking such detail,,,

  8. Thanks Zhen for taking the time to summarize the conference for us, very informative.

  9. Zhen, your summary of the NYC meeting is truly wonderful. I’m sorry that I didn’t meet you there; I was too busy being shy. Your writing is great, but what interests me most is the facts as you presented them.

    There was only one additional fact that seemed very significant to me that you didn’t report on: There is now a planned clinical trial that will directly compare HU and Interferon. That trial is exactly what is needed, since there is so much dissent about which is the better treatment, and the fact is that right now, no one knows. Dr. Barbui labeled this trial as a
    “Randomized Trial of Pegylated Interferon Alfa-2a versus Hydroxyurea for the treatment of high risk PV and high risk ET patients (MPD-RC trial).” As an ET patient, I’m going to age out of the low risk category in 9 years, and I’m hoping there will be some useful data out of that trial that will inform my decision about treatment.

    I went to Dr. Barbui’s session on ET, and it was superb. His talk was titled “Current European Leukemia Net Recommendations on the management of Essential Thrombocythemia.” He presented new treatment guidelines for ET that are the result of a panel of 21 European and American experts (including most of the doctors we know), combining to review recent research and results of the big MPN hospitals treatment records, and coming up with a consensus of treatment guidelines. Notably, “consensus” was defined as 80% of the doctors agreeing. Here are two highlights of the talk:

    For ET patients who are defined as high risk, HU is deemed to be the first-line treatment, with IFN or Anagrelide as a second-line treatment.

    Although Aspirin is effective in PV patients in preventing major bad events like strokes and heart attack, the situation is not the same for low-risk ET patients. Patients with platelet count over 1000 x 10^9/L had an increased risk of major bleeding. JAK2+ patients taking aspirin had an increased risk of venous thrombosis. And patients with cardiovascular risk factors taking aspirin had an increased risk of arterial thrombosis. The recommendation is to limit the use of aspirin in low-risk ET patients, and use it only with great caution. “For each patient an individual assessment of thrombotic and hemorrhagic risks should be made.”

  10. Peggy Frederick said:

    Greetings Zhen: So appreciate your having taken the time to put this piece together for the benefit of those who may not have had the opportunity to attend the Symposium on the 2nd of November. It was great meeting you at the Symposium, but unfortunately, we did not have enough time to really have a substantive chat. Regret that. We may bump into each other at another meeting. Hope so. Your synopsis of the meeting was comprehensive, entertaining, and informative . . . who could ask for more? In addition, the fact that you are obviously a good writer added a nice touch to the recap of some very technical information. Hopefully, we will be able to keep in touch via FB or some other internet venue. Best of luck with the course you choose to treat (or not to treat) the MF.

    Warmest regards,
    Carl & Peggy Frederick
    Nutley, NJ

  11. Thank you, Zhen, for such a terrific review of the NY Symposium. Your writing and perspective is informative, amusing and interesting.

    The pretzel at the Hot Dog stand outside the Univrsity Club was a disappointment. I remember from my youth thst they were salty and crispy on the outside and soft on the inside and tasty. It was not.

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