Category Archives: Advocacy

Understanding Thrombocytosis in MPN Patients: What You Need to Know

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For patients living with myeloproliferative neoplasms (MPNs), the term “thrombocytosis” often arises. While thrombocytosis—an elevated platelet count—is a hallmark of some MPNs like essential thrombocythemia (ET), it can also appear in other forms of MPNs or even due to unrelated secondary causes. Understanding the different contexts in which thrombocytosis occurs is key to effective management and improving quality of life.

Thrombocytosis in MPNs: A Common Feature

1. Essential Thrombocythemia (ET)

  • Primary Cause of Thrombocytosis: In ET, the overproduction of platelets is driven by genetic mutations such as JAK2, CALR, or MPL in the stem cells of the bone marrow.
  • Platelet Levels: Platelet counts in ET are persistently elevated, often exceeding 450,000/μL, and can reach over 1,000,000/μL.
  • Risk of Complications: ET-associated thrombocytosis increases the risk of blood clots (thrombosis) and bleeding due to dysfunctional platelets.

2. Polycythemia Vera (PV)

  • Secondary Thrombocytosis: PV primarily involves elevated red blood cell counts, but platelet counts are often high as well. This occurs because of the overactivity of the bone marrow, commonly linked to the JAK2 mutation.
  • Complications: In PV, elevated platelets further amplify the risk of clotting, especially when combined with high red blood cell counts.

3. Myelofibrosis (MF)

  • Variable Platelet Counts: In early stages of MF, thrombocytosis may occur due to hyperactive bone marrow. However, as the disease progresses and fibrosis (scarring) of the bone marrow develops, platelet counts often drop (thrombocytopenia).
  • Implications: Elevated platelets in early MF contribute to the overall risk of thrombosis but are usually less prominent than in ET or PV.

Thrombocytosis in MPNs vs. Reactive Thrombocytosis

MPN patients may also develop reactive thrombocytosis, where platelet levels rise due to an external trigger rather than the disease itself. This is important to differentiate, as the treatment approach varies.

Causes of Reactive Thrombocytosis in MPN Patients:

  • Infection: Common colds, bacterial infections, or systemic inflammation.
  • Iron Deficiency: Iron depletion, often seen in PV due to phlebotomy or blood loss, can elevate platelet counts.
  • Surgery or Trauma: Any significant physical stress can temporarily increase platelet production.
  • Inflammatory Conditions: Co-existing autoimmune diseases or inflammatory processes.

Managing Thrombocytosis in MPN Patients

For MPN patients, managing thrombocytosis involves addressing both the underlying condition and associated risks:

1. Medications to Control Platelet Levels

  • Low-Dose Aspirin: Reduces the risk of clotting in patients with high platelet counts and cardiovascular risks.
  • Cytoreductive Therapy: Drugs like hydroxyurea or anagrelide may be prescribed to reduce platelet counts in high-risk patients.
  • JAK Inhibitors: For conditions like PV or MF with thrombocytosis, drugs like ruxolitinib target the underlying JAK2 pathway.

2. Monitoring and Prevention

  • Regular Blood Tests: Monitoring platelet counts and clotting markers is crucial.
  • Lifestyle Modifications: Staying active, avoiding smoking, and maintaining a healthy weight can help reduce clotting risks.
  • Avoiding Triggers: Identifying and managing secondary causes like iron deficiency or inflammation can prevent exacerbation.

3. Managing Complications

  • Clotting Risks: Thrombocytosis in MPNs increases the risk of strokes, heart attacks, and deep vein thrombosis (DVT). Prompt treatment of symptoms like chest pain, shortness of breath, or limb swelling is essential.
  • Bleeding Risks: Paradoxically, MPN patients with thrombocytosis may experience bleeding due to abnormal platelet function, such as nosebleeds, gum bleeding, or gastrointestinal bleeding. Report unusual bleeding to your healthcare provider immediately.

Living with Thrombocytosis as an MPN Patient

Thrombocytosis in the context of MPNs requires long-term management, but there are steps you can take to improve your quality of life:

  • Stay Informed: Learn about your specific MPN and its implications for thrombocytosis.
  • Build a Support Network: MPNs are rare conditions. Connecting with support groups or online communities can provide emotional support and practical advice.
  • Communicate with Your Care Team: Keep an open dialogue with your hematologist, and don’t hesitate to ask about treatment options, clinical trials, or lifestyle recommendations.

Thrombocytosis in MPN patients is more than just a high platelet count—it’s a complex condition with significant implications for your health. Understanding the nuances of your condition is essential for effective management, whether it’s caused by essential thrombocythemia, polycythemia vera, or reactive triggers. By working closely with your healthcare team and staying proactive in your care, you can navigate the challenges of thrombocytosis and live a fuller, healthier life.

Persian Gulf War Service Linked to High Rates of Myeloproliferative Neoplasms

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by Mary Anne Dunkin | Sep 15, 2024

WASHINGTON, DC — A study of almost a half-million veterans has found for the first time a link between environmental exposures during military service and the development of myeloproliferative neoplasms (MPNs).

MPNs—including polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF)—are a group of rare, heterogeneous and acquired clonal stem-cell disorders, which lead to uncontrolled proliferation of myeloid cells and complications including arterial and venous thrombosis, bleeding, cardiovascular disease and potentially the development of leukemia. The study’s findings could open MPNs to be recognized as presumptive conditions under the Promise to Address Comprehensive Toxics (PACT) Act, suggested Maneesh R. Jain, MD, one of the study’s leaders.

Jain, a hematologist/oncologist at the Washington, DC VAMC, became intrigued with a possible connection between military exposures and MPN when three of his female patients who had served in the Korean War were diagnosed with MPNs. All three believed their disease was related to exposure to Agent Orange (a tactical herbicide used by the U.S. military for the control of vegetation), as were a number of other veterans they communicated with thought an MPN advocacy group.

To better understand a possible connection, Jain and colleagues at Georgetown University and George Washington University, including hematology/oncology fellow Andrew Tiu, MD, turned to the DoD and VA Infrastructure for Clinical Intelligence (DaVINCI). DaVINCI is an electronic network that provides a consolidated view of electronic medical record data for both service members and veterans.

Their retrospective cohort study, published in the American Journal of Hematology, included 65,425 Korean War era veterans, 211,927 Vietnam War era veterans, and 214,007 Persian Gulf War era veterans from Jan. 1, 2006, to Jan. 26, 2023. Veterans with MPN, thrombosis, bleeding, and cardiovascular risk factors were identified through ICD-9 and -10 codes. Illinois was selected as the state of residence, as it best mirrored the demographics of the entire U.S. cohort in terms of age, race, ethnicity and educational attainment according to the American Community Survey from the U.S. Census Bureau.1

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Risk of myeloproliferative neoplasms among U.S. Veterans from Korean, Vietnam, and Persian Gulf War eras

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July 18, 2024

Andrew Tiu, Zoe McKinnell, Shanshan Liu, Puneet Gill, Martha Antonio, Zoe Shancer, Nandan Srinivasa, Guoqing Diao, Ramesh Subrahmanyam, Craig M. Kessler, Maneesh Jain

Abstract

The Promise to Address Comprehensive Toxics (PACT) Act expanded U.S. Veterans’ health care and benefits for conditions linked to service-connected exposures (e.g., Burn Pits, Agent Orange). However, myeloproliferative neoplasms (MPN) are not recognized as presumptive conditions for Veterans exposed to these toxic substances. This study evaluated the development of MPN among U.S. Veterans from the Korean, Vietnam, and Persian Gulf War eras. This retrospective cohort study included 65 425 Korean War era Veterans; 211 927 Vietnam War era Veterans; and 214 007 Persian Gulf War era Veterans from January 1, 2006, to January 26, 2023. Veterans with MPN, thrombosis, bleeding, and cardiovascular risk factors were identified through ICD-9 and -10 codes. Veterans from the Persian Gulf War era had the highest risk of developing MPN compared with Veterans from the Korean and Vietnam War eras, hazard ratio (HR) 4.92, 95% confidence interval (CI) 4.20–5.75 and HR 2.49, 95% CI 2.20–2.82, both p < .0001, respectively. Vietnam War era Veterans also had a higher risk of MPN development compared with Korean War era Veterans, HR 1.97, 95% CI 1.77–2.21, p < .0001. Persian Gulf War era Veterans were diagnosed with MPN at an earlier age, had higher risks of thrombosis and bleeding, and had lower survival rates compared with Korean War and Vietnam War era Veterans. This study reinforces evidence that environmental and occupational hazards increase the risk of clonal myeloid disorders and related complications, impacting overall survival with MPN. Limitations include the inability to confirm clonality and fully verify deployment and exposure status.

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Patient reported outcomes among U.S. Veterans living with myeloproliferative neoplasms

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Authors: Natasha Mathur, Andrew Chua  Tiu, Ann Brazeau, Natalie Giocondo, Puneet Gill, Shanshan Liu, Guoqing Diao, Ramesh Subrahmanyam, Craig M Kessler, and Maneesh Rajiv Jain

Abstract

Background: Myeloproliferative Neoplasms (MPNs) profoundly affect patients’ well-being, necessitating effective symptom relief. Patient-reported outcomes are pivotal for care improvement, aligning with Centers for Medicare and Medicaid Services priorities. This study aims to assess MPN symptom burden among U.S. Veterans, examining risk levels, thrombo-hemorrhagic complications, medication use, information access, care, and service-connected benefits.
Methods: Conducted as a cross-sectional survey from December 1, 2023, – January 22, 2024, all Veterans in the MPN Advocacy Education & International database were recruited. Symptom burden was assessed using the validated MPN Symptom Assessment Form Total Symptom Score (MPN SAF TSS), with descriptive statistics and T-tests employed for analysis.
Results: Of 61 respondents, 88% were male, 92% Caucasian, 88% served during the Vietnam war. The mean age was 73±7, with MPN diagnosis at 64±9 and a mean duration of 44±10 years from service-connected exposure. Key findings included 56% Polycythemia Vera, 26% Essential Thrombocythemia, 16% Primary Myelofibrosis, 70% JAK2, 10% CALR, 3% MPL, 14% unaware of their mutation status, and 34% unaware of MPN risk status. Seventeen (28%) underwent phlebotomy, 64% used Hydroxyurea, 29% Ruxolitinib, and 3% Interferon. Fatigue and itching were the predominant and severe symptoms (Table), with 43% reporting no symptom improvement. Mean MPN SAF TSS of all respondents was higher than prior study by Emanuel et.al. which adjusted for MPN subtype (36.4 vs. 21.6, p<0.0001). Twelve (20%) reported bleeding and 10% reported venous thrombosis after MPN diagnosis, while 13% reported arterial thrombosis before MPN diagnosis. Twenty-two (36%) are not seeing an MPN specialist and 61% are not seeing a hematologist at their local Veterans Affairs Medical Centers. Twenty-three (38%) did not seek a second opinion while 43% do not have a caregiver. Respondents turn to MPN organizations (75%), physician offices (74%), Google Search (49%), Webinars (41%), and Facebook (23%) for information. Five (8%) received service-connected disability benefits.
Conclusions: Veterans with MPNs endure a substantial symptom burden, surpassing previous studies, signifying suboptimal management. Limited access to care persists, necessitating enhanced support. However, self-reporting bias and potential limitations in activity assessment must be acknowledged. Efforts to enhance MPN awareness, specialized care, and support mechanisms are imperative for comprehensive management.

Advocacy: PDABs on Our Radar

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In response to rising drug costs, some states are creating Prescription Drug Affordability Boards, or PDABs, that have varying degrees of oversight. PDABs are tasked with determining reasonable drug prices based on things such as: if the price affects a patient’s access to a drug, if there are other drugs proven to do the same thing for a cheaper price, and what the drug manufacturer charges. Additionally, special consideration is to be taken for drugs that treat rare conditions, and input from patient communities is supposed to be included in the decision-making process.

Colorado was one of the first states to create a PDAB back in 2021 and they are currently reviewing 5 drugs, one of which is an orphan drug used by cystic fibrosis patients. Each state decides how they will select the drugs for review. Other states with PDABs are Maine, Maryland, Minnesota, New Hampshire, Oregon, and Washington. States that introduced PDAB legislation in 2023 are Connecticut, Michigan, New Jersey, New Mexico, Rhode Island, Vermont, and Virginia.

As a patient advocacy and education organization, we want to ensure that our MPN community is informed about PDABs and how they could impact your access to MPN drugs. We also want to make certain that the MPN patient voice is central to any board if an MPN drug comes up for review. Most importantly, we want to make sure that every MPN patient benefiting from an MPN drug has access to it.

We want to hear from you! Are you in a state with a Prescription Drug Affordability Board? If so, do you know how your board chooses which drugs will be reviewed or which drugs are currently up for review? Let us know by contacting Ann Brazeau at abrazeau@mpnadvocacy.com.
Stay tuned as we learn more about PDABs and make plans to ensure the MPN voices are heard!

Abstract WP249: Risk for Ischemic and Hemorrhagic Stroke is Increased in Veterans Exposed to Agent Orange and Those With Myeloproliferative Neoplasms

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Natasha Mathur, Andrew Tiu, Zoe McKinnell, Puneet Gill, Martha Antonio, Shanshan Liu, Guoqing Diao, Ramesh Subrahmanyam, Craig M Kessler and Maneesh R Jain

Abstract

Agent Orange (AO) is a dioxin containing defoliant and carcinogen used in the Korean and Vietnam War. There is limited evidence of the association between AO exposure among Veterans and stroke. Stroke is not yet part of the list of presumptive conditions according to the Promise to Address Comprehensive Toxics (PACT) Act which provides Veterans and their survivors disability compensation for conditions arising from exposure to AO. Myeloproliferative Neoplasms (MPN) are uncommon etiologies of stroke but whether AO exposure increases incidence of stroke in MPN has not been described.

Utilizing the Veterans Affairs Informatics and Computing Infrastructure (VINCI) database, a case-control study was performed from 1/1/2006 – 1/26/2023 on the Veterans from Illinois, the state most representative of the US population. ICD-9 and -10 codes identified Veterans with stroke and MPN. AO exposure was verified on the Veterans’ service duration and location. Qualitative data were compared by chi-square tests.

Among 586,555 Veterans from Illinois, there were 15,455 ischemic stroke (IS), 1,593 hemorrhagic stroke (HS), 2,752 MPN, and 59,393 with AO exposure. Among MPNs, there were 237 IS (41 with AO) and 26 HS (3 with AO). IS and HS were associated with AO exposure, OR 1.34 95% CI 1.28-1.41, p<0.0001, and OR 1.20 95% CI 1.03-1.39, p=0.02, respectively. MPN is associated with IS and HS, OR 3.52, 95% CI 3.08-4.03, and OR 3.54, 95% CI 2.4-5.23, both p<0.0001, respectively. There is no significant association with AO exposure among Veterans with MPN with stroke. Among non-MPN Veterans with AO exposure, there was an earlier median age of IS and HS, 67 vs. 70 and 67 vs. 71, both p<0.0001. There was no difference in median age of stroke among MPN Veterans with or without AO exposure. There were no differences with rates of hypertension, hyperlipidemia, diabetes, smoking, heart failure, and pulmonary hypertension among MPN Veterans with stroke with and without AO exposure.

In conclusion, there is an association of AO exposure with IS and HS with an earlier onset among those exposed. There is a strong association between MPN and stroke independent of AO exposure. The biologic plausibility of endothelial dysfunction and accelerated atherosclerosis from AO exposure warrants further investigation.

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Mindfulness Minute: Finding Fire Inside

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By Natalie Giocondo

When the winter winds are howling and the bones are deeply chilled, remember that you can always create a fire on the inside. Perhaps the dropping temperatures have a calming effect on some common MPN symptoms like itching or night sweats, but the darker days certainly can take a toll on the emotional body-especially if the blues are already something that you struggle with.

Winter is a season of stillness, so an excess of inertia is not surprising; however, moving less can also manifest as feelings of depression. Depression is no stranger to the MPN community, in a well-known 2017 study conducted by Dr. Claire Harrison and her colleagues, around 61% of participants indicated feelings of depression. To balance out the dark coldness of winter, we need to create fire (agni) within.

Both yogis and scientists know that the body is in a constant state of flux, reacting to external and internal inputs and trying to maintain balance. From the yogic perspective, we are perpetually moving between too much energy (rajas) and not enough energy (tamas), trying to find balance (sattva).

Tips on Creating Fire Inside

● Avoid eating meats, stale or fermented foods, and underripe fruits and vegetables. These foods slow our digestion and only perpetuate inertia or stagnation in the body.

● Eat six smaller meals to promote consistent blood sugar, and keep the internal embers burning.

● Drink warm beverages like herbal teas, hot cider, or warm water with lemon.

● Savor the experience of eating and drinking turning mealtime into a daily meditative practice that cultivates gratitude and combats feeling low.

● If you see the sun, bundle up and get your face in it, even if only for a few moments.

● Use candles and fireplaces to supplement artificial light after the sun sets.

● Do exercises and yoga practices that engage the solar plexus and create energy and warmth in the body.

Join us online on Thursday, December 14th from 12:00-12:30pm EST where we will do an asana (yoga poses) practice that focuses on the solar plexus and will give you tools to combat depression and create a fire inside. This practice will require a yoga mat or a rug to practice on and will incorporate seated and kneeling poses. Until then, stay warm and imagine your inner summer.

 

Agent Orange exposure shows higher risk for myeloproliferative neoplasm development

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June 15, 2023

By David Statman

In this video, Andrew Tiu, MD, discussed results from a study regarding the link between Agent Orange and myeloproliferative neoplasms, which he presented at ASCO Annual Meeting.

Tiu, a hematology/oncology fellow at MedStar Georgetown University Hospital, noted that the study showed an increased likelihood for developing myeloproliferative neoplasms after exposure to Agent Orange, as well as several other conditions.

“We look forward to looking more into the cardiovascular outcomes with myeloproliferative neoplasms, as well as Agent Orange exposure,” Tiu said.

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A Veteran’s Story: The Frustrations of Filing a Claim with the VA

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By Wayne E.

MPN Patient and Vietnam Veteran Wayne E.

I served in the USAF Security Service, 6924th Security Squadron, stationed in Da Nang, Vietnam for one year (1970-1971) and was exposed to the deadly Agent Orange/Dioxin. In 2007, after a simple pre-op blood test, I was diagnosed with essential thrombocythemia (ET). Upon further study I was told I had an incurable, but manageable, blood cancer, coupled with a gene mutation (JAK2). The word cancer scared me. I had never heard of ET and I was at a loss for what to do. I didn’t know where to go next. After much reading about these potentially deadly diseases, I found out I was one of many Vietnam Veterans who had an MPN.

In 2011, I filed my first claim with the VA. Until this filing, I was unable to get any substantial information from my primary care physician (PHP) or my hematologist/oncologist, as to what may have caused or contributed to my ET. They knew virtually nothing about Agent Orange. I contacted the National Institutes of Health, The Centers for Disease Control, and as many online medical sites as possible, all ending with a bigger question mark. Nothing could be explained to satisfy my inquiry.

It was by chance that I connected with a most remarkable group, MPN Advocacy and Education International. I could never thank them enough for the compassion and the understanding they extended to me.

After my initial rejection from the VA, I filed three more times and each time I was denied because MPNs are not on the “presumptive” list of Agent Orange-related illnesses. The same message I kept getting was I needed “clinical rationale” to support my claims. My doctors have not been able to provide me with this needed information. I don’t know what to do today. I understand there are many Vietnam vets that have won their appeals and now get benefits, but there are many others who were not approved and just gave up. I don’t plan to give up.

To my fellow Vietnam Veterans who may be dealing with one of these MPNs, don’t give up. If you have been denied, file an appeal. There is hope, comfort, and assistance available. With the help of MPN Advocacy and Education International.

 Learn more about filing a claim with the VA

 Learn more about Veterans and MPNs