March 2025
Category Archives: Blood Cancer
Safety and Efficacy of Busulphan Based on Dosing Patterns in the Real‐World Management of Myeloproliferative Neoplasms
March 2025
Abstract
Myeloproliferative neoplasms (MPNs), such as polycythaemia vera (PV), essential thrombocythemia (ET) and myelofibrosis (MF), are primarily treated by managing blood counts to reduce the thrombotic risk using cytoreductive agents. Busulphan, an oral alkylating agent, has been historically used for MPN management due to its myelosuppressive effects, but concerns about its risk of leukaemic transformation have limited its use.
The role of psychosocial adjustment in the quality of life of patients with myeloproliferative neoplasms
March 7, 2025
A.A.M. Eppingbroek, L. Lechner, E.C. Bakker, M.D. Niijkamp, M.A. de Witte, C.A.W. Bolman
Abstract
Purpose
Methods
Results
Conclusion
Hematologic Cancers Among Patients With Type 2 Diabetes Prescribed GLP-1 Receptor Agonists
March 6, 2025
Omer S. Ashruf, BS1Jasmin Hundal, MD, MS, MPH2Ali Mushtaq, MD3; et al
Type 2 diabetes (T2D) and obesity have been identified as independent risk factors for various cancers, including hematologic cancers.1 Glucagon-like peptide–1 receptor agonists (GLP-1RA) have emerged as an effective treatment, offering glycemic control, weight reduction,2 and immune modulation,3 and are associated with lower cancer risk, specifically solid tumors.4 However, the association of GLP-1RA with hematologic cancers remains unexplored. This study aims to compare the risks of hematologic cancers in patients with T2D treated with GLP-1RA compared with metformin and insulin.
Evolution of WHO diagnostic criteria in “Classical Myeloproliferative Neoplasms” compared with the International Consensus Classification
March 4, 2025
Jürgen Thiele, Hans Michael Kvasnicka, Umberto Gianelli, Daniel A. Arber, Ayalew Tefferi, Alessandro M. Vannucchi, Tiziano Barbui & Attilio Orazi
Abstract
A lively discussion persists regarding the diagnostic criteria for essential thrombocythemia (ET), primary myelofibrosis (PMF) and polycythemia vera (PV), particularly in relation to early/pre-fibrotic myelofibrosis (pre-PMF), a disease entity initially introduced in 2001 by the 3rd edition of the World Health Organization (WHO) classification. The definition and criteria used to diagnose pre-PMF have been progressively modified over time. The most update definition of pre-PMF can be found in the International Consensus Classification (ICC) published in 2022. An updated largely similar definition is also incorporated in the recently published 5th edition of WHO classification (2024). Diagnostic criteria for ET have undergone changes up to 2016/17 for the revised 4th edition of the WHO. In particular the threshold value for platelets were lowered and the important discrimination between “true” and “false” ET (in reality pre-PMF) been widely acknowledged. To avoid misdiagnose in early phase PV, the criteria for gender-adjusted thresholds for hemoglobin/ hematocrit have been lowered and the identification of an appropriate bone marrow (BM) morphology was upgraded as a major diagnostic criterion. Given the prominent role of morphology in MPN-related diagnostic algorithms, the diagnostic adequacy of the BM biopsy (sample procurement and proper laboratory handling) as emphasized in former WHO editions and in the ICC, was not addressed by the WHO 5th. The essential role of genetic markers is recognized by both classifications. A comparison between the revised 4th edition WHO classification and the ICC versus the WHO 5th reveals no significant differences, with the exception of the occurrence of leukoerythroblastosis in pre-PMF considered by the latter as one of the minor diagnostic criteria which seems unwarranted. In contrast to the revised 4th edition, the majority of the microscopic images used for the WHO 5th due to their low magnification and poor technique, do not highlight the diagnosis differences among these entities.
Genetic Testing Breakthroughs in Blood and Lymph Cancers
February 28, 2025
Hematopoietic and lymphocytic neoplasms (HLNs) are a diverse group of malignancies affecting blood and lymphatic systems, with outcomes varying from manageable conditions to fatal diseases. Traditional classifications rely on morphology, karyotyping, and fluorescence in situ hybridization (FISH). However, recent advancements in next-generation sequencing (NGS) allow simultaneous genetic profiling of multiple genes, enhancing diagnostic precision and therapeutic strategies. This review examines key molecular applications in diagnosing and managing HLNs, addressing current challenges and proposing solutions to optimize clinical utility.
Chronic Myeloid Leukemia (CML)
CML, historically identified by leukocytosis, is characterized by the BCR::ABL1 fusion gene resulting from the Philadelphia chromosome translocation. This oncogenic fusion drives aberrant tyrosine kinase activity, promoting unchecked proliferation. The introduction of imatinib, a targeted tyrosine kinase inhibitor (TKI), revolutionized CML treatment, leading to normalized white blood cell (WBC) counts within months. However, resistance mutations necessitate molecular monitoring via quantitative PCR, FISH, and karyotyping, ensuring optimal therapeutic adjustments.
Molecular Applications in BCR::ABL1-Negative Myeloid Neoplasms
Certain myeloid neoplasms, such as chronic neutrophilic leukemia (CNL) and chronic eosinophilic leukemia (CEL), lack the BCR::ABL1 fusion gene but exhibit distinct genetic markers like CSF3R mutations in CNL. Classical myeloproliferative neoplasms (MPNs) include polycythemia vera, essential thrombocythemia, and primary myelofibrosis, driven by JAK2, MPL, or CALR mutations. The application of NGS enables comprehensive mutational profiling, aiding accurate diagnosis and prognostication.
Building a Foundation of Trust in Patients With MPNs
For oncology nurses and APPs caring for patients with chronic conditions like MPNs, fostering a comfortable environment begins with active listening that extends beyond clinical data, an expert said.
Understanding the patient’s life outside the exam room—their sources of joy and their personal challenges—is essential for providing holistic care. Given the nature of MPNs, these providers often develop long-term relationships with patients, sometimes seeing them more frequently than they see their own families. Therefore, prioritizing the establishment of trusting relationships through deeper patient engagement is paramount for optimizing care and support throughout the patient’s journey.
Oncology Nursing News’ sister publication, CURE, spoke with Kathryn Johnson, DNP, MSc, FNP-BC, at the in-person MPN Heroes event to learn more about how connections like these can really benefit patients with MPNs.
Johnson is a Clinical Program Manager at Icahn School of Medicine at Mount Sinai New York.
Understanding Thrombocytosis in MPN Patients: What You Need to Know
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.
Transforming Care With Collaboration, Individualized Treatment, and Novel Therapies
Author(s): Laura Joszt, MA
December 20, 2024
Patients with chronic hematologic malignancies are living for decades, especially with new treatments, making it an important time to shape value-based treatments being offered to these patients, said Jennifer Vaughn, MD, during a fireside chat at the Cleveland Regional Institute of Value-Based Medicine (IVBM) event hosted by The American Journal of Managed Care.
Vaughn, a hematology specialist specializing in myelodysplastic syndromes at The Ohio State University, was joined by Akriti Jain, MD, a hematologist at Cleveland Clinic, to discuss quality care initiatives in rare hematological disorders.
“One of the main things that we talk about these days is individualizing care, right? Not every patient is the same,” Jain said. “So, when I see a patient with myelofibrosis in clinic, the first question is: What are they presenting with?” If a patient has the typical symptoms of myeloproliferative neoplasms (MPNs), a JAK inhibitor is probably the right way to go, she said. If they don’t have those symptoms but they have anemia or thrombocytopenia, then a little more investigation is needed.In the polycythemia vera space, there are also a number of agents now available that can lead to a reduced risk of progression in the future. Vaughn explained that when she sees a younger patient, they now have the opportunity to take aspirin and go to the doctor for routine phlebotomies and labs or a treatment that they can manage and can limit time away from work and their kids.
“That’s been, now, a really interesting discussion in that patient population for me, because there are many of my patients who have actually opted to go on therapy,” she said. “We all think of phlebotomy as this very low-risk, easy [procedure] to undergo, but phlebotomy is just a real…pain for them. They can’t spend the time away.”
She added that “time toxicity” is being considered more and more, which is a way to evaluate how much time patients spend having to engage in their health care treatments.
Prognostic Implications of Pulmonary Hypertension in Myeloproliferative Neoplasms and Predictors of Hematologic Progression
Orly Leiva, MD, Steve Soo, MD, Nathanial Smilowitz, Harmony Reynolds, Binita Shah, Samuel Bernard, Michelle Hyunju Lee, MD, Chi-Joan How, MD, and Gabriela S. Hobbs, MD