Відео

Some of the slides say essential thrombocytopenia ???

Thank you! I learned a lot from this talk. Dr Scandura is always _very_ informative.

Marvelous clinical and professional approach hope u LL continu

The common complications of myeloproliferative neoplasms (MPNs) that are most concerning are progression to myelofibrosis and transformation to acute leukemia. The rates of progression vary by MPN subtype, with 10-20% of primary myelofibrosis patients progressing to leukemia compared to 2-3% in polycythemia vera and less than 1% in essential thrombocythemia. Other important complications in MPNs relate to symptoms, quality of life issues, as well as thrombotic and vascular complications, which are the most common causes of morbidity and mortality. Thrombotic problems can occur in the arterial or venous circulation, in both large and small vessels. In the venous system, deep vein thrombosis (DVT) and pulmonary embolism (PE) are common. DVT involves blood clot formation in leg veins which can break off and travel to the lungs, causing a PE. Intra-abdominal clots also occur, including in the hepatic and portal veins (bud-chiari syndrome and portal vein thrombosis). Arterial clots can occur in peripheral, cerebrovascular and coronary arteries, causing tissue damage through lack of oxygen. Small vessel disease also occurs, evidenced by erythromelalgia which causes burning pain and redness in the extremities. This is relieved by aspirin, showing the critical role of platelets in microvascular complications. The mechanism of thrombosis likely involves abnormal platelet function, compounded by dysfunctional white cells and red cells. Endothelial cells lining all blood vessels may also contribute. Further research at the cellular level is required to fully elucidate mechanisms. Currently, only aspirin has good evidence for reducing thrombotic complications in MPNs (ECLAP trial). Risk factors for thrombosis include: older age, prior thrombosis, extreme thrombocytosis, high white cell count, JAK2 mutation, coexisting cardiovascular risks, bone marrow fibrosis, and inherited thrombophila. A 3-tiered risk classification system helps guide management. Better biomarkers and genomic medicine approaches may improve individual risk prediction. In summary, common complications negatively impacting quality of life and survival in MPNs include leukemic transformation, progression to myelofibrosis, and thrombohemorrhagic events. The pathogenesis of clotting is not fully understood, with abnormal platelet-vessel wall interactions likely a factor. Only aspirin has clear efficacy. Further research on the cellular mechanisms will enable development of newer, targeted antithrombotic therapies for MPNs.

Myelofibrosis is a progressive bone marrow disorder where fibrotic tissue replaces normal bone marrow. Patients may initially present with mild anemia, thrombocytosis, and minimal splenomegaly. Over time they develop symptomatic splenomegaly, progressive cytopenias, and constitutional symptoms that impact quality of life. Median survival ranges from 3-20 years depending on mutational profile. The only curative therapy is allogeneic stem cell transplant. Treatment options include ruxolitinib to reduce spleen size and symptoms, erythropoiesis stimulating agents for anemia, low-dose aspirin to reduce thrombosis risk, and prednisone during ruxolitinib interruptions. Survival is improving with better patient selection for transplants and new drugs targeting inflammation like telomerase inhibitor imetelstat and BET inhibitor CPI-0610 that reduce symptoms and transfusions. Essential thrombocythemia (ET) is an indolent MPN with too many platelets. The goal of therapy is minimizing thrombosis and bleeding risk, reducing symptom burden, and preventing transformation to myelofibrosis (MF) or acute myeloid leukemia (AML). Survival is better than in PV or MF but vascular events are still the main cause of morbidity/mortality. Risk-stratified treatment options include observation, low-dose aspirin, hydroxyurea, pegylated interferon, anagrelide, and ruxolitinib. Hydroxyurea and interferon have clinical trial evidence supporting use in high-risk patients. Interferon may have better molecular responses, especially in CALR-mutated patients. Ruxolitinib improves ET-related symptoms but not response rates. New therapies like telomerase inhibitor imetelstat are being studied. Thrombosis and bleeding remain significant problems in MPNs, directly linked to pathobiology and outcomes. Arterial clots usually occur with underlying atherosclerosis while venous clots occur due to abnormal blood flow and hypercoagulability. Unique to MPNs is a propensity for abdominal vein thrombosis. Acquired von Willebrand disease explains some bleeding tendencies. Beyond blood counts, the proinflammatory state created by mutant MPN cells activates blood cells and endothelium, creating a prothrombotic circulation. Recently Cloneal hematopoiesis of indeterminate potential (CHIP) mutations have been connected to increased coronary artery disease risk, even in healthy older individuals. We now recognize MPNs as an independent risk factor for atherosclerosis but the mechanisms require further study. For patients, preventing thrombosis remains paramount. In conclusion, myelofibrosis has seen substantial progress in prognostication allowing better patient selection for curative stem cell transplant. Median survival continues to improve with second line therapy options like imetelstat and CPI-0610 that target diseased bone marrow and inflammation. Essential thrombocythemia remains troublesome for its thrombotic and bleeding complications, however hydroxyurea and interferon have proven benefits in high-risk patients. New insights into the clonal origins of cardiovascular disease in MPNs and clonal hematopoiesis warrant further investigation to clarify thrombosis and bleeding mechanisms in these patients. Continued clinical trial enrollment is critical for developing better tolerated treatments that modify the natural history of these chronic malignancies.

Introduction The 11th patient symposium on myeloproliferative neoplasms (MPNs) was organized by the Cancer Research & Treatment Fund to provide scientifically-based information to MPN patients. Speakers included leading experts in the field. Key topics covered: - The role of inflammation in MPNs - Using next generation sequencing for diagnosis and treatment - Interferon prolonging myelofibrosis-free and overall survival in polycythemia vera Inflammation in MPNs - Chronic inflammation drives progression of the MPN malignant clone from early stage to advanced myelofibrosis. - Inflammation elicits blood clots, atherosclerosis, and other cancers in MPNs, similar to other inflammatory diseases. - MPNs arise from an inflammatory state and can be viewed as a model of inflammation-driven cancer. Consequences of Inflammation in MPNs - Increased fractures possibly due to inflammation-mediated bone loss - Chronic kidney disease progression akin to diabetes, showing MPNs elicit systemic inflammatory effects - Age-related macular degeneration risk illustrating inflammatory co-morbidities - Increased co-occurrence of inflammatory bowel disease and MPNs Additional Effects - Higher risk of second cancers before and after MPN diagnosis, including skin, lung, and urinary tract - Smoking as a novel MPN risk factor, fitting with links between inflammation and other myeloid neoplasms - MPNs associated with increased thrombosis risk, driven by cytokine signaling mutations Perspectives - Many citizens harbor MPN mutations without overt disease. They have constant thrombosis risk that causes serious complications years before diagnosis. - Earlier diagnosis and treatment is needed to modify underlying disease and attenuate inflammation before end organ damage occurs. Goals of Future Therapies - Target the malignant clone - Address concurrent inflammation - Prevent clonal evolution and reduce thrombosis risk - Achieve minimal residual disease status as a platform for curative therapies like vaccination Interferon Therapy Considerations - Works "hard and long" to induce durable control of disease - The only drug that targets MPN stem cells - Analysis shows treating early lowers mutant allele burden faster - Personalized treatment allows allele burden monitoring to guide therapy Combination Therapies - Adding the JAK inhibitor ruxolitinib "quells the fire" of inflammation that drives disease - Statins have hypothetical additive anti-inflammatory, anti-thrombotic activity worth studying - Combining interferon, ruxolitinib, and potentially statins may profoundly improve outcomes Future Outlook - Screen target populations for molecular evidence of MPNs - Intervene early with interferon to eradicate malignant clones - Study adjunctive anti-inflammatory medications - Achieve minimal residual disease status in preparation for curative therapies The dream: Curing MPNs by detecting and eliminating every last malignant cell before end organ damage occurs. Next Generation Sequencing in MPNs Driver mutations: - Initiate and propagate malignancy by increasing cytokine signaling - Mainly target JAK-STAT pathway, causing overproduction of myeloid lineage cells - Represent acquired mutations not passed to offspring Detection methods: - PCR-based assays identify known hotspot mutations - Next generation sequencing (NGS) reads millions of DNA sequences in parallel - NGS quantifies mutation burden across multiple genes Using NGS data: - Helps establish MPN diagnosis by identifying driver mutations - Distinguishes MPN subtypes based on distinct drivers - Predicts prognosis using integrated clinical and genetic risk models Interpreting NGS: - Co-occurring mutations frequently present and modify disease features - Research is defining implications of many new gene mutations - Changes in mutant allele burden don't have defined significance yet Conclusions: - NGS outpaces current clinical understanding of extended gene panels - Oncogenetics fosters better personalized therapies - Having genetic data enables discussing disease considerations Polycythemia Vera and Interferon Disease background: - Myeloproliferative neoplasm causing red blood cell overproduction - Driven by JAK2 mutations leading to thrombosis and myelofibrosis risk - Hydroxyurea or interferon used in high-risk patients Well Cornell approach: - Considers interferon over hydrea due to disease-modifying effects - Retrospectively assessed interferon impact on 470 PV patients Key findings: - Interferon substantially prolonged myelofibrosis-free survival - Overall survival was also extended by interferon therapy - Benefit increased with duration of interferon treatment Conclusion: - Interferon has a profound disease-modifying effect in PV - It produces durable remissions and prevents fibrosis progression - Should be strongly considered across PV risk groups

THANK YOU. ET PATIENT TRIPLE NEGATIVE. DX AT 25 YRS. IM 60 NOW. NOW HAVING SYMPTOMS. THAT ARE DIFFICULT

Garbage treatments. No real answers. So sad

Pls make a video on Erythrocytosis. Thank you.

If only my hemotology/onocologist was interested in research and learning anything.

This video is from 7yrs ago. There is a lot of new drugs today.

I am looking for a hemathologist in Bucharest, Romania who understands and applies what dr Jerry L Spivak is talking about. So far ,all 6 doctors i went to see suspect ET and are asking for bone marrow biopsy(which I refused) so they can tell the difference between ET and PV and start me on hydoxiurea , although i have no symptoms. I am 32 years old and my blood count is 800k to 1 mil platelets ,all other blood values are normal.I still have to check for Jak2, Calr, Mpl.These videos taught me more than the doctors I saw could explain, they all sounded like robots citing WHO criteria and getting mad at me for refusing the bone marrow biopsy saying only with it they can give me treatment. If you know of such doctor please let me know.Thank you

I am looking for a hemathologist in Bucharest, Romania who understands and applies what dr Jerry L Spivak is talking about. So far ,all 6 doctors i went to see are asking for bone marrow biopsy(which I refused) so they can tell the difference between ET and PV and start me on hydoxiurea , although i have no symptoms. I am 32 years old and my blood count is 800k to 1 mil platelets ,all other blood values are normal.I still have to check for Jak2, Calr, Mpl.These videos taught me more than the doctors I saw could explain, they all sounded like robots citing WHO criteria and getting mad at me for refusing the bone marrow biopsy saying only with it they can give me treatment. If you know of such doctor please let me know.Thank you

gOOD day i have a friend who got diagnosed with this disease. Can you please help him . He live in the Philippines he has no work and he is taking care of his mother who is bed ridden. He has no money for his check up he hadn't been to a specialist before

I have PV since 2011 at the same time I have liposarcoma. Are these two health problems related with each other? I just had my 3rd surgery of liposarcoma very recently. If there are available studies on these two health issues, i am willing to participate. Thanks

Thank you so much Dr Dagba for curing me of polycythemia vera😭🙏🏼

Thank you so much Dr Dagba for curing me of polycythemia vera😭🙏🏼

I increased my vitamin C intake from 1g to 4g daily, using absorbable forms of the vitamin (liposomal) and my experience is a drop of ~ 75% in itching. I suffer from polycythaemia and itching has always been a big issue for me, affecting my lifestyle, since I react severely to temperature changes and contact with water. I assume the condition increases histamine transport, as it increase basophil production? This would explain why the vitamin C is reducing itching, since it is recommended for histamine control.. See this video for example: ua-cam.com/video/A97H9QUlZiA/v-deo.html

Great Video! Such an imporant topic to shed light on.

Just started my journey , found the abnormal blood counts now about to confirm with other blood tests , I'm a 68 year old male , so I started to research this and found Dr. Silver and glad I did

Great info , I think I was diagnosed with PV just days ago , I'm 68 , male , and I am going back for more blood tests to confirm it , I think I had this for the last 25 years , it would explain my things that have happened to me during that time , I seemed to have self threated myself with aspirin all that time too , lately I got an O2 meter and have noticed a lower level at times , now this would explain that , not a lot but but a few points down , I will let you all know what my next blood test say , one will be the Jak 2 Gene test

What percentage of population with the JAK2V617F mutation do not go on to develop a myeloproliferative neoplasm?

Ich möchte Dr. Ediale ein großes Dankeschön aussprechen, der ein Heilmittel für alle Arten von Krankheiten hat, wie Herpes, Gürtelrose, Diabetes, Ulser, Lippenherpes, HIV, HPV, idiopathische Lungenfibrose, kontaktieren Sie ihn, wenn Wenn Sie eine dieser Auswirkungen oder Krankheiten haben, hat er seine Kräutermedizin verwendet, um meinen Vater von idiopathischer Lungenfibrose zu heilen Er war fast am Aufgeben, als ich online ein Zeugnis eines anderen Patienten sah, den er von Fibrose geheilt hatte. Zuerst dachte ich, es sei eine Fälschung, bis ich ihn testete und herausfand, dass er ein echter und zuverlässiger Arzt war. Kontaktieren Sie ihn per E-Mail: dredialeherbalhome2@gmail.com oder rufen Sie ihn an / whatsapp (+2348109213597) Vielen Dank Doktor Ediale auf UA-cam.

Wow I can see how much work it is to keep HGB that low just to counteract the amount of platelets.

Awesome video! They should teach the importance of mental health in schools. Keep it up!

I was dx in 01 with ET (platelets over 1 million) then morphed in MF in o5. I’ve had two blood clots, Ischemic Colitis (then Dr. put me on Plavix), and a blockage in the intestines from too much scar tissue from prior laparoscopic surgeries (adhesions). In 2011, had my Gallbladder removed and one week later they found a Thymoma Tumor in the Thymus Gland and I had a Thymectomy (stage one, malignant). In 2018, had to take an ambulance to ER and they had to do emergency surgery, removal of my Appendix and when they took out my appendix they found a Neuroendocrine Carcinoid Tumor in my Appendix (stage IIA, malignant). And in 2019 I went to the ER for severe stomach pains and they said I had a 70% blockage in my Celiac Artery. I went to a top Vascular Surgeon at Mt. Sinai and he did some tests and said normally they would put a stent in, but due to my high risk and when he did the sonogram he said the body sometimes has a way of healing itself, instead of my blood going into my main artery, it has gone in another way, so its going where it is suppose to. We just have to keep an eye on it. I’ve been through a lot since dx with ET in 01. I had grade 2 fibrosis, and I have Osteoarthritis and severe joint and bone pain. My counts are ok. My hgb is 10.1 and my leukocytes is very low. Otherwise, I look like a picture of perfect health. Tired a lot, itch and the joint & bone pain. I see Dr. Macaranhas at Mt. Sinai. Just wanted to say I had two tumors and one blood cancer, Myelofibrosis. Stacey H. Long Island, NY

Where can I sign up for one of the studies? Dr. Srdan Verstovsek, MD, PhD?

There are other ways to go and i am suppose to just wait but my iron is so low. I think they think that magically my iron will go up so they can do a phlebotomy, with no other plan. This is a cancer that one can live a long time BUT GET A DOCTOR THAT IS NOT KNOWLEDGABLE and you are playing with your life. I know if something happens, it will be their mismanagement but only i will know and i will be dead at that point. So i have done enough research to know that waiting is NOT the way to go. The problem now is after 3 weeks of trying to just get the specialist (where in Maryland there are only 5 that i know of) and trying to get an apt has been INSANE.....lm so frustrated. The quality of my life has plumeted while "WAITING" and they honestly seem to be UNCONSERNED...... Strange enough my brother was diagnosed before me, at the exact same age as me, but he was diagnosed with a bone marrow biopsy JAK2 neg and for years they couldn't figure out why my blood numbers were a mess but not until my brother had a stroke and was eventually diagnosed did my doctors look at this possibility....I was diagnosed 18 months later with the JAK2 mutation. I moved to his doctor but my case is more complicated, not only due to past medical circumstances but now due to the iron deficiency but i still constantly worry about him now with this doctor. Im just so frustrated and tired of the lack of caring in the medical field. In myexperience i have had too many crazy situations because doctors that are confronted with my case find it too complicated but Polycythemia Vera has clarified so much of the problems. Im so frustrated and tears have become a part of my day.... AND ABOVE ALL PERSAVERANCE! GET A SPECIALIST....DO NOT GET A DOCTOR THAT DOESN'T HAVE EXPERIENCE!!! I WISH I HAD KNOWN THAT FROM THE BEGINING i am convinced that my care is why my life and symptoms have now gotten out of control ! Best of Luck to all!

Mine is platelets why do they keep going up they are 565

Cancer is the most profitable business in the world and the cure for Cancer is banned! Vitamin b17 is so toxic to Cancer!

Mr. Hans Hasselbalch - a great person and great professional, who kindly helped me very much with his advises, not knowing me personally!

While these people blather on about their nonsense accomplishments, it is interesting to note almost no progress has been made in cancer "cure" statistics. In fact from 1 in 9 in the 70's to 1 in four, and now the information that 1 in two people will get cancer in their lifetime, according to the NCI's own statistics. The money is in treatment, even though many of these treatments will kill the patient faster than the cancer will. It's sad, but when when more people are making a living from cancer than there are dying from it, a cure is the last thing the cancer industry wants. It is a fact that a number of promising cancer treatment alternatives to poisonous radiation, poisonous chemotherapy, and invasive surgury are suppressed, mainly because natural cures can't be patented, so there is no money in it. People still listen to, and believe the bullshit line "We will conquer cancer." It's just a sales pitch. If it wasn't, the cancer industry wouldn't be putting a miniscule percentage of the money they raise into research. It's a racket, plain and simple. Watch "The Cancer Report" for more insight into how this industry operates. I have researched the cancer industry for many years, and not one thing that I've just said can be disproven. If you are going to comment on my post, use some actual facts, and real information to back up your refutation. Name calling will only display your own ignorance. Thank you.

Differences by age diagnosed, like in their 30’s?

I have ET. I’m going into my 6th year with this MPN. I am 66 years old. No hx of cardio vasular disease or thrombosis. Only my age. I have Jak 2 kinase. My question is, can I get a stem cell transplant? I am a twin. My twin brother will definitely donate stem cells. When I get to that stage. Thank you, excellent video! Originally my platelet count was 1,150,000. Dr put me on Hydrea and Anagrelide. I failed Hydrea. It didn’t fail me, I failed it. I have been on Anagrelide 0.5mg q.i.d. I do the 0.5 because it is cheaper. I had breast cancer and was treated with high doses of radiation. I believe that is what caused my ET! I realize I may be older, but I will have a new husband. I want as much time with him as I can get. I want to live and love longer! 🙏🤞🏼🎄

Thank you for that very interesting video. Can you provide more information on what particular yoga exercises are recommended for those who have MPNs? Thanks

I have ET, 65 y/o, diagnosed in 2014. Platelets were 1,250,000! Hydroxuria was a failure. Anagrelide working well. Last count was 187,00 I had breast ca in 2014. Platelets started during radiation treatments. Thank you so much Dr!

I so wish mainstream medecine would investigate functional medical therapies to improve patients lives ......

I am on a keto diet.... bulletproof diet life style .... Raw fresh juices .... cannabis oil to sleep deeply .... Weight lifting program... advanced holosync meditation program .... Ozone therapy ... Nutritional infusions ..... Hormone replacement therapy..... Etc etc the difference all these protocols make are remarkable .....

As a point of sharing my experience with Mylofibrosis and the functional medical protocols I have applied into my lifestyle program in order to mitigate all the negative symptoms .... I am very disturbed by the lack of information ..... I have made it my no. 1 passion to find ways to create a quality of life ... Not expecting this from my medical team ..... Yes Jakavi saved my life but without all the various natural healing protocols I have implemented I would not be thriving as I am at 77. I was diagnosed with secondary Mylofibrosis .... Had the other 2 ... First diagnosed with thrombocytosis penia in my early 50s.

Thanks for intersting information )..

just started researching this for my wife......HELP?????

learning much

I have Polycythemia Vera and I have a World Authority Doctor at Johns Hopkins Hospital in Maryland. To avoid clotting, stroke and heart attack you must get your blood checked regularly. This speaker just mentioned my doctors name who is Dr. Spivak. I was diagnosed 2/26/02 in my home town which is 5 hours south of Maryland. The safest levels are a Hemoglobin of a 12 but not over and a HCT of a 36. Focus is mainly on your Hemoglobin. If your Hemoglobin rises above a 12 then a pint of blood needs to drawn off you. Because we have a high platelet count we have to keep our HGB and HCT low. According to this speaker it seems that things have changed so it maybe time for me to go back to Johns Hopkins and have a visit with my World Authority Doctor because this man tells my Hematologist here at home how I'm to be treated.

my daddy is a MPN patient. .. Please how can you help my daddy ... This is my email please reply me sir .. mekalabharaths@gmail.com

my daddy is a MPN patient. .. Please how can you help my daddy ... This is my email please reply me sir .. mekalabharaths@gmail.com

my daddy is a MPN patient. .. Please how can you help my daddy ... This is my email please reply me sir .. mekalabharaths@gmail.com

If HCT is a value derived from RCB and MCV, what are the safe, acceptable ranges of values for both RCB and MCV? And how to keep RCB and MCV within those acceptable ranges?

would it be possible to use prm151 jakafi combination in severe cases and then say after 1.5yrs revert to IFN?

Question: what is the desired outcome from this design of expt and what seems great interesting work? Does it need further studies eg to verify / refine? Is it ready to roll out to to the clinic now so that types of disease can be stratified, and appropriate treatment applied based on severity of disease from 10 gene testing. If not why not? Is the testing not available widely, if not why not? Another question when will pRM 151 trials start, given it takes time to work (~1.5 years). will it go straight to phase 3 , will it be combined with eg jakafi in trials?