Category Archives: Blood Cancer

MPN Word of the Month: Hematocrit

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Hematocrit is a key measurement in hematology that represents the proportion of blood volume occupied by red blood cells (RBCs). Expressed as a percentage, it provides crucial insights into an individual’s red blood cell mass and overall blood health. Typically, hematocrit levels are assessed through a routine blood test, often as part of a complete blood count (CBC).

In the context of myeloproliferative neoplasms (MPNs)—hematocrit plays a significant role in diagnosis and management. Polycythemia vera, essential thrombocythemia, and myelofibrosis each affect blood cell production but in different ways.

  1. Polycythemia Vera (PV): One of the indicators of PV is an elevated hematocrit level. In PV, the bone marrow produces an excess of red blood cells, leading to a high hematocrit. This can increase how thick the blood is (viscosity), potentially causing complications such as blood clots, strokes, or heart attacks. Regular monitoring of one’s hematocrit is essential for managing PV and assessing the effectiveness of treatments aimed at reducing the risk of these complications.
  2. Essential Thrombocythemia (ET): While ET primarily involves elevated platelet counts, a high hematocrit may also be observed due to secondary effects or overlapping features with other MPNs. Management focuses on controlling platelet levels to prevent thrombotic events, but monitoring hematocrit remains important for comprehensive disease management.
  3. Primary Myelofibrosis (PMF): In PMF, hematocrit levels may be low due to the replacement of bone marrow with fibrous tissue, leading to anemia. The disease’s progression can cause varying hematocrit levels, which are crucial for tracking disease progression and response to treatment.

In summary, hematocrit is more than just a routine blood test value; it is a vital indicator in the diagnosis, treatment, and management of myeloproliferative neoplasms.

Immunofluorescence microscopy on the blood smear identifies patients with myeloproliferative neoplasms

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July 17, 2024

Carlo Zaninetti, Leonard Vater, Lars Kaderali, Carl C. Crodel, Tina M. Schnöder, Jessica Fuhrmann, Leonard Swensson, Jan Wesche, Carmen Freyer, Andreas Greinacher & Florian H. Heidel

Myeloproliferative neoplasms (MPN) are a group of clonal stem cell disorders with heterogeneous clinical presentation [1]. Due to the risk of severe thromboembolic complications and disease progression, the early recognition of an MPN prior to the appearance of clinical complications is clearly warranted to facilitate early pharmacologic intervention [2,3,4]. Detection of the somatic mutations by genotyping has become an essential part of the diagnostic work-up of suspected subjects, as well as of the risk stratification after the diagnosis of MPN has been confirmed [5]. However, in many parts of the world molecular testing is barely affordable.

We have established an immunofluorescence microscopy (IF)-based method for platelet phenotyping on the peripheral blood smear [6]. This method has been proven to be highly efficient in the diagnosis of diverse hereditary platelet disorders by recognizing disease-specific changes of cell structures, including alterations of leukocytes and red blood cells (RBC) [7, 8]. Major advantages of this approach are the need of small amounts of blood (<100 μL) and the possibility to send the blood films by regular mail even long distances.

It is well-known that morphology of peripheral blood cells is also often altered in MPN [9, 10]. However, due to different methods and the heterogeneity of the patients’ populations, results are difficult to compare.

In the present study, we aimed at assessing platelet phenotype using our IF method in a cohort of patients diagnosed with MPN. The study has been registered in the German Clinical Trials Register (DRKS-ID: DRKS00032588). Three German reference centers for diagnosis and treatment of MPN took part in the study: Internal Medicine C, University Medicine Greifswald; Internal Medicine 2, University Hospital Jena; and Hematology, Hemostasis, Oncology and Stem Cell Transplantation, Hannover Medical School, Germany. The study protocol was approved by the institutional review boards of all centers. Patients or their legal guardians signed written informed consent to the investigation, which was conducted according to the Declaration of Helsinki. Healthy controls were enrolled among blood donors at the Institute for Transfusion Medicine, University Medicine Greifswald, Germany.

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Predictors of symptom scores in myeloproliferative neoplasms: A real-world retrospective cohort study

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Muhammad Ali Khan, Syed Arsalan Ahmed Naqvi, Irbaz Bin Riaz, and Jeanne M. Palmer

Abstract

Background: Although high symptom burden indicates poor survival and informs treatment decisions, little is known about the impact of demographic, clinical, and laboratory features on total symptom score (TSS) in patients with myeloproliferative neoplasms (MPN).
Methods: Patients with MPN (polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF)) were identified from the retrospective chart review. TSS, individual symptom scores (fatigue, early satiety, abdominal discomfort, inactivity, concentration problems, fever, night sweats, itching, bone pain, weight loss), demographic characteristics (race, ethnicity, age, gender), clinical features (time since diagnosis, depression status, obesity status, spleen size), laboratory results and season at the time of visit were recorded from the clinical encounter when index assessment of TSS was performed for each patient. Normality was assessed using visual inspection of data distribution, whereas multicollinearity was assessed using various inflation factors. A univariable regression followed by a multivariable regression analysis was conducted using a backward selection approach. A p-value <0.05 indicated a statistically significant association of a given feature with TSS.
Results: The chart review identified 252 patients (PV: 78; ET: 81; MF: 93). Mean age was 59 (SD: 17.7), 67 (SD: 13.0), and 68 (SD: 10.9) years for ET, PV, and MF respectively. Most patients were white (PV, MF: 92%; ET: 83%) and females (ET: 75%; PV: 60%; MF: 53%). The TSS of patients was highest with PV (mean: 18.5; SD: 16.9) followed by MF (mean: 18.1; SD: 15.4) and ET (mean: 14.3; SD: 15.9). Fatigue was the most reported symptom whereas the least reported symptoms were fever and weight loss. Univariable regression analyses showed depression (B: 17.7; p=0.02), female gender (B: 10.6; p=0.01), platelet count (B: 0.03; p=0.03), and hemoglobin (Hb) (B: -2.6; p=0.01) in PV patients, depression (B: 19.8, p=2×10-5) in ET patients and depression (B: 11.0, p=0.03), white blood cell (WBC) count (B: 0.2; p=0.01), neutrophil count (B: 0.3, p=0.01), and non-neutrophil WBC count (B: 0.6; p=0.02) in MF patients to have significant association with TSS. Multivariable regression analyses (Table) showed Hb (B: -2.5; p=0.01) and platelet count (B: 0.02; p=0.03) in PV patients, depression (B: 19.7; p=2×10-5) in ET patients and depression (B: 12.3, p=0.01) and WBC count (B: 0.3; p=0.002) in MF patients to have a significant association with TSS.
Conclusions: Depression in ET and MF and low Hb in PV were identified as significant drivers of symptom burden. Identifying and managing patients with these comorbidities could improve their quality of life with a potential survival benefit.

Masarova on the Latest Updates Across Myeloproliferative Neoplasms

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Lucia Masarova, MD

Lucia Masarova, MD, PhD, assistant professor, Department of Leukemia, Division of Cancer Medicine, The University of Texas MD Anderson Cancer Center, discusses some of the latest updates across myeloproliferative neoplasms (MPNs).

She discusses some significant studies, including the TRANSFORM-1 and MANIFEST-2 studies (NCT04472598; NCT04603495), and emerging therapeutic approaches, like combining ruxolitinib (Jakafi) with other agents, such as pelabresib (CPI-0610) and navitoclax.

Transcription:

0:09 | We are waiting for the top-line results of the phase 3 studies of the frontline ruxolitinib and the navitoclax vs ruxolitinib and placebo, as well as the top-line results of the other combo of ruxolitinib and pelabresib vs ruxolitinib and placebo. Both of those, the TRANSFORM-1 and MANIFEST-2 study studies, are highly expected results. They are coming to see whether we can move from the monotherapy of a JAK inhibitor to the combination of arms of both of the agents. That will expand the field, hopefully, and we will see where it is going to go. [There are] excellent results for spleen control that are exciting, the symptoms will need to be worked out, so we will see where it goes.

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Dr. Guo Develops Test Promising Answers for Cancer Patients

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Cancer researcher, Belinda Guo, knows that one of the biggest issues for people with myeloproliferative neoplasms (a group of rare blood cancers), is not knowing if the cancer will progress.

Dr Belinda Guo and her team at the University of Western Australia’s Translational Cancer Pathology Laboratory have invented a new blood-based test that detects specific changes in the blood of patients diagnosed with myelofibrosis.

With funding from Cancer Council WA, they are now working with clinicians to assess the blood of individuals who have undergone a bone marrow transplant to see if the transplant has worked.

We sat down with Belinda to hear more about her research project. Read the full interview below.

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Follow-Up Needs for Blood Cancer Survivors May Determine Best Type of Provider

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Laura Joszt, MA

For patients with blood cancers, follow-up care consisting of management of psychosocial consequences, promotion of a healthy lifestyle, and disease prevention may be better addressed by primary care physicians (PCPs) than oncologists, according to a study published in Cancer Medicine.

The study, conducted in Germany, found most survivors of blood cancers were receiving care at a university hospital and a minority were actually being care for by community oncologists or PCPs. The researchers evaluated follow-up care received by survivors from the University Hospital of Essen using a questionnaire.

“Given the favorable prognosis of many types of blood cancer, there is a wealth of information about long-term treatment side effects, secondary diseases, and quality of life. How and by whom follow-up care is delivered, however, remains largely unexplored,” the authors noted.

Follow-up can be provided in different ways. In one model, oncologists provide follow-up care related to cancer and general practitioners provide other health care at the same time. In another model, survivors of cancer are transferred to PCPs for continued care. In a more complex model, oncologists and general practitioners have complementary roles.

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A Review About the Assessment of the Bleeding and Thrombosis Risk for Patients With Myeloproliferative Neoplasms Scheduled for Surgery

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Mihaela Andreescu • Bogdan Andreescu

Published March 12, 2024

Abstract

Myeloproliferative neoplasms (MPNs) present a unique challenge in surgical management due to their inherent predisposition to both bleeding and thrombosis. MPNs are a heterogenous group of acquired clonal conditions. The three classic MPNs are essential thrombocythemia (ET), myelofibrosis (PMF), and polycythemia vera (PV). All subtypes of MPN are associated with both thrombotic and bleeding complications. There are four risk categories for thrombosis in MPN patients: age, thrombosis history, and JAK-2 mutation. They are further classified as very low, low, intermediate, and high risk. The genetic landscape of MPN is fascinating and complex like all myeloid disorders. Bleeding risk can be assessed through leukocytosis, thrombocytosis, acquired von Willebrand syndrome (AVWS), and a previous history of bleeding in a patient. Risk assessment and perioperative management are important aspects of improving the quality of life and preventing complications in surgeries. Preoperative management includes a risk assessment of venous thromboembolism, use of appropriate pharmacological treatment, platelet count control, and correction and cardiovascular risk factors. This review summarizes the assessment of bleeding and thrombosis risk for patients with MPNs scheduled for surgery. Furthermore, this review discusses various tools that can be used to identify MPN patients at risk of thrombosis prior to surgery.

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AYA With MPN Face Psychological Needs That Are Poorly Understood

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Laura Joszt, MA

Up to 20% of patients with myeloproliferative neoplasms (MPN) are adolescent and young adults (AYAs) and the population is growing; however, there is a paucity of data on the psychological needs of AYAs with MPN, according to a systematic scoping review published in Leukemia & Lymphoma.1

Previous research has shown AYAs with cancer can be overlooked2 and there is little information on how this population does during treatment and survivorship,3 but they can face unique psychosocial issues that impact their quality of life.4

“While the MPN AYA group generally reports a similar symptom burden to the adults with MPN, very little is known about the psychological impact and management of AYA with MPN,” the authors wrote. “Understanding psychosocial issues patients with MPN face are critical given the demands of cancer, and its treatment is often directly counter to the developmental needs of the age group.”

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Dr Jennifer Vaughn: Patients With MPN, MDS Should Discuss Long-Term Priorities Upfront

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Laura Joszt, MA
Justina Petrullo
With most patients with myeloproliferative neoplasms (MPNs) and myelodysplastic syndromes (MDS) experiencing long-term, chronic disease, it’s important to discuss their priorities and set up the relationship with their providers upfront, explained Jennifer Vaughn, MD, assistant professor in the division of hematology at The Ohio State University Comprehensive Cancer Center – James Cancer Hospital and Solove Research Institute.

Incyte Launches The Unseen Journey to Elevate the Hidden Impact of Myeloproliferative Neoplasms (MPNs) on Patients’ Lives through Generative AI

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February 29, 2024

– The Unseen Journey brings to life the often-misunderstood impact of common myeloproliferative neoplasm (MPN) symptoms through AI-generated images developed from the words and experiences of real patients

WILMINGTON, Del.–(BUSINESS WIRE)–Incyte (Nasdaq: INCY) today announced the launch of The Unseen Journey, a program that brings to life the hidden emotional and physical toll of myeloproliferative neoplasms (MPNs), a group of rare, chronic and progressive blood cancers. Through the use of generative artificial intelligence (AI), the stories and experiences of MPN patients were transformed into unique images to help them show their health care team and their loved ones the significant impact of their MPN symptoms.

The Unseen Journey highlights the stories of people living with MPNs who were asked to describe their symptoms and how they impact their lives in their own words. As each patient described their symptoms and experiences, generative AI tools transformed their words into images that visually depict the patient’s most burdensome symptoms. The resulting images provide a vivid look at the reality of living with an MPN.

“MPN symptoms can be difficult to recognize and describe and every patient’s experience is different, which can sometimes create a challenge for patients, their loved ones and their health care teams to understand the impact of the condition to daily life,” said Ann Brazeau, CEO and Founder, MPN Advocacy and Education International. “These AI-generated images paint a vivid picture of what it is like to live with an MPN, and I hope they will help create a new level of awareness and empathy for those with these conditions.”

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