Category: Cancer and neoplasms

Around 16,000 people die in the UK from blood cancer every year, but the warning signs can be hard to spot. Blood cancer is the third biggest cancer killer and there are more than 100 different types of the disease, but some of them start with vague and easy-to-miss symptoms. As is the case with all cancers, early diagnosis is crucial to improve treatment outcomes. The more well-known variations of blood cancer include leukaemia, lymphoma and myeloma, but there’s also myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPN), as Blood Cancer UK explains. All cases of blood cancer are caused by mutations in DNA within blood cells, which cause the cells to start behaving abnormally, as the Mirror reports. But what are the signs and symptoms of this potentially-devastating disease? Read more: Doctor Michael Mosley shares ‘critical’ food to lose weight and keep it off According to Blood Cancer UK, there are 11 common red flags to watch out for. These include: Unexplained weight loss Unexplained bruising or bleeding Lumps or swellings Breathlessness Drenching night sweats Infections that are persistent, recurrent or severe Unexplained fever (37.5°C or above) Unexplained rash or itchy skin Pain in your bones, joints or abdomen Fatigue that doesn’t improve with rest or sleep Paleness (pallor) – the skin under your lower eyelid looks white rather than pink. One in 19 people will develop blood cancer, so if you have just one symptom that you can’t explain, it’s advised to book an appointment with your GP as soon as possible. If you suddenly feel very unwell at any time, get medical help by calling 999 or going to A&E. It is not fully understood why someone will develop blood cancer, but the disease has been associated with genetic as well as environmental factors. Yale Medicine explains that smoking, radiation exposure and exposure to certain chemicals have all been linked to increased risk of some types of blood cancers. The health website states: “Epstein-Barr virus, HIV and human T-cell lymphoma/leukaemia virus infections are also risk factors for developing lymphomas and leukaemias.” Leukaemia is one of the most widely-known types of blood cancers which affects blood cells in the bone marrow, usually white blood cells. It occurs when the body creates too many abnormal white blood cells and interferes with the bone marrow’s ability to make red blood cells and platelets. Blood cancer is caused by changes in the DNA within blood cells. As part of Blood Cancer Awareness month, which takes place every September, Blood Cancer UK has urged people to break the silence surrounding blood cancer by saying its name. A new campaign is encouraging people with blood cancer to qualify that their individual condition is a type of blood cancer to help raise vital awareness. in the video, actor and broadcaster Stephen Fry said: “76% people aren’t told what they have is blood cancer when they’re diagnosed, they end up finding out on Google or not at all. And they’re missing out on being part of a supportive community. And that’s why I’m asking when you’re speaking about these conditions to say blood cancer.” People with experience of chronic lymphocytic leukaemia, multiple myeloma, chronic myeloid leukaemia, and those MPNs are all featured in the film. Aimee Togher, 23, is another face of the campaign and was just 22 when she found a lump on her neck. She was later diagnosed with stage 2 Hodgkin-lymphoma, a form of blood cancer. She said: “Getting a blood cancer diagnosis was a complete shock. Not everyone gets told this. Many people don’t know what blood cancer actually is. It isn’t just one cancer – it’s so many different types. I was lucky that I knew lymphoma was a type of cancer. The ‘c word’ was never actually mentioned by my doctor. We need to spread awareness of blood cancer, what it is, who’s affected by it, so that we can bring people together.” Helen Rowntree, chief executive of Blood Cancer UK said: “Few realise that blood cancer is amongst the top five most prevalent cancers in the UK, and even fewer know that leukaemia, lymphoma and myeloma are all types of blood cancer. By breaking the silence, raising awareness, we hold the power to reduce the harm blood cancer causes. Let us unite to make a difference in the lives of those battling these conditions.”

An increase in asthma-associated emergency department visits during and after exposure to wildfire smoke suggests a need for planning and public health strategies to reduce this exposure, especially in regions of the United States where wildfire smoke exposure was previously uncommon. This study was published in a CDC Morbidity and Mortality Weekly Report. “Community preparedness and appropriate and prompt response are crucial to reduce wildfire smoke exposure and morbidity,” wrote the researchers of the study. “Recommended actions include assessing a possible health care utilization surge related to wildfire smoke exposure.” Between April 30, 2023, and August 4, 2023, millions of adults and children across the United States were exposed to wildfire smoke originating from wildfires in Canada. Particulate matter, generally particles ≤2.5 μm in aerodynamic diameter (PM2.5), is known to exacerbate cardiovascular, metabolic, and respiratory conditions. However, little is known about the health implications of prolonged episodes of high concentrations of wildfire smoke. Advertisement Data from the National Syndromic Surveillance Program (NSSP) assessed numbers and percentages of asthma-associated emergency visits on days with wildfire smoke compared with days without. The data included approximately 6000 emergency visits, which represented 76% of all eligible facilities in the United States, with 4317 facilities representing 85% of all NSSP facilities. Wildfire smoke days were defined as days with PM2.5 elevated Air Quality Index (AQI) of 100 or greater, corresponding to the air quality categorization, “Unhealthy for Sensitive Groups.” Additionally, observed daily numbers and percentages of asthma-associated emergency visits were stratified by region and age groups: 0-4 years; 5-17 years; 18-64 years, and 65 years and older. Observed visits were defined as the number of visits reported on a given day and expected visits were calculated using anomaly detection algorithms. The data showed that during the 19 days of wildfire smoke, overall asthma-associated emergency visits were 17% higher than expected across all age groups and regions but was most common among individuals ages 18 to 64 years. Additionally, increased emergency visits were more common on days with a higher percentage of air quality monitors reporting PM2.5 concentrations. Furthermore, larger increases in asthma-associated emergency visits were observed I regions that experienced higher numbers of continuous wildfire smoke days and among individuals ages 5 to 17 years and 18 to 64 years. The researchers acknowledge some limitations to the study, including that this report cannot be directly attributed to the increase in AQI to wildfires in Canada and may have excluded patterns of wildfire smoke health effects in subregional areas. Despite these limitations, the researchers believe the study suggest how health excess exposure to wildfire smoke is associated with an increase in asthma-associated emergency visits. Furthermore, the researchers advocate for measures to reduce and prevent these exacerbations through emergency respond planning and public health communication strategies, especially among older individuals. “Jurisdictions interested in using syndromic surveillance to monitor the public health implications of wildfire smoke might consider using asthma as an initial indicator to develop strategies to reduce exacerbations and reach populations at increased risk for both exposure and adverse health effects,” wrote the researchers. “Expanded monitoring of health conditions, including cardiopulmonary-related ED [emergency department] visits, might also improve understanding of the severity of the impact of wildfire smoke on health outcomes and amplify prevention efforts to reduce these exacerbations.” Reference Asthma-associated emergency department visits during the Canadian wildfire smoke episodes – United States, April– August 2023. Centers for Disease Control and Prevention. August 24, 2023. Accessed August 31, 2023. https://www.cdc.gov/mmwr/volumes/72/wr/mm7234a5.htm?s_cid=mm7234a5_e&ACSTrackingID=USCDC_921-DM111326&ACSTrackingLabel=This+Week+in+MMWR%3A+Vol.+72%2C+August+25%2C+2023&deliveryName=USCDC_921-DM111326#F1_down.

Around 16,000 people die from blood cancer every year in the UK, making it the third biggest cancer killer – but what are the signs and symptoms of the devastating disease? There are more than 100 different types of blood cancer, with most being variations of leukaemia, lymphoma and myeloma, but they can also include myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPN), explains Blood Cancer UK. All cases of blood cancer are caused by mutations in DNA within blood cells, causing the cells to start behaving abnormally. Warning signs of the disease can be hard to spot as symptoms are often vague, but early diagnosis is vital to ensure better treatment outcomes. According to Blood Cancer UK, there are 11 common red flags to watch out for. These include: Unexplained weight loss Unexplained bruising or bleeding Lumps or swellings Breathlessness Drenching night sweats Infections that are persistent, recurrent or severe Unexplained fever (37.5°C or above) Unexplained rash or itchy skin Pain in your bones, joints or abdomen Fatigue that doesn’t improve with rest or sleep Paleness (pallor) – the skin under your lower eyelid looks white rather than pink. One in 19 people will develop blood cancer, so if you have just one symptom that you can’t explain, book an appointment with your GP as soon as possible. If you suddenly feel very unwell at any time, get medical help straight away by calling 999 or going to A&E. Want to get the latest health news direct to your inbox? Sign up for the Mirror Health newsletter HERE Unexplained bruising or bleeding is a blood cancer warning sign ( Getty Images) Who is at risk of blood cancer? It is not fully understood why someone will develop blood cancer, but the disease has been associated with genetic and environmental factors. Yale Medicine explains that smoking, radiation exposure, and exposure to certain chemicals have all been linked to increased risk of some types of blood cancers. The health site writes: “Epstein-Barr virus, HIV and human T-cell lymphoma/leukaemia virus infections are also risk factors for developing lymphomas and leukaemias.” What is the difference between blood cancer and leukaemia? Leukaemia is a type of blood cancer that affects blood cells in the bone marrow, usually white blood cells. Leukaemia occurs when the body creates too many abnormal white blood cells and interferes with the bone marrow’s ability to make red blood cells and platelets. Blood cancer, meanwhile, is caused by changes in the DNA within blood cells. Blood Cancer Awareness month As part of Blood Cancer Awareness month – which takes place every September – Blood Cancer UK have urged people to break the silence surrounding blood cancer by saying its name. In a campaign video, those with lived experience – alongside actor Stephen Fry – encourage people with blood cancer to qualify that their individual condition is a type of blood cancer, to help raise crucial awareness. Stephen said: “76% people aren’t told what they have is blood cancer when they’re diagnosed, they end up finding out on google or not at all. And they’re missing out on being part of a supportive community. And that’s why I’m asking when you’re speaking about these conditions to say blood cancer.” Drenching night sweats is a red flag of blood cancer ( Getty Images) People with experience of chronic lymphocytic leukaemia, multiple myeloma, chronic myeloid leukaemia, and those MPNs are all featured in the film. Aimee Togher, 23, is one of the faces of the campaign and was just 22 when she found a lump on her neck, she was later diagnosed with stage 2 Hodgkin-lymphoma, a form of blood cancer. She said: “Getting a blood cancer diagnosis was a complete shock. Not everyone gets told this. Many people don’t know what blood cancer actually is. It isn’t just one cancer – it’s so many different types. I was lucky that I knew lymphoma was a type of cancer. The ‘c word’ was never actually mentioned by my doctor. We need to spread awareness of blood cancer, what it is, who’s affected by it, so that we can bring people together.” Aimee has five tumours in three places, with one being over 5 cm and close to her heart and lungs, which makes treatment riskier. She’s now completed intensive chemotherapy. “I wasted summers stressing over how big my arms looked in vest tops, if my hair and skin looked greasy and now, I’m living with the side effects of chemotherapy, blood cancer is humbling,” she said. “The road to recovery is far from easy, but with unwavering determination and the support of loved ones, I’ve taken each step with the hope of better days.” Helen Rowntree, Chief Executive of Blood Cancer UK said: “Few realise that blood cancer is amongst the top five most prevalent cancers in the UK, and even fewer know that leukaemia, lymphoma and myeloma are all types of blood cancer. By breaking the silence, raising awareness, we hold the power to reduce the harm blood cancer causes. Let us unite to make a difference in the lives of those battling these conditions.”

What is POEMS syndrome?SymptomsPolyneuropathyOrganomegalyEndocrinopathy/edemaMonoclonal gammopathySkin changesOther symptomsPathophysiologyDiagnosisTreatmentReferences Further reading ‘POEMS syndrome’ refers to polyneuropathy, organomegaly, endocrinopathy/edema, monoclonal gammopathy, and skin changes. Taken together, these symptoms reflect a paraneoplastic disease that, in rare cases, arises in patients with plasma cell neoplasms. ​​​​​​​Image Credit: Bangkok Click Studio/Shutterstock.com What is POEMS syndrome? A plasma cell neoplasia is a benign or malignant disease defined by the overproduction of plasma cells. The three types of plasma cell neoplasms include monoclonal gammopathy of undetermined significance (MGUS), plasmacytoma, and multiple myeloma. POEMS syndrome can develop in patients aged 50 or older in extremely rare cases of plasma neoplastic diseases. Notably, men are at a 1.5 times greater risk of POEMS syndrome than women. Due to the rarity of POEMS syndrome, it is often difficult to recognize. As a result, the incidence of this paraneoplastic syndrome is likely underrepresented, leading to challenges in its management. Symptoms As indicated in its name, POEMS syndrome is associated with several distinct signs and symptoms. Polyneuropathy Polyneuropathy can cause patients with POEMS syndrome to experience numbness, tingling, and/or weakness in their extremities, particularly in the legs and hands. Patients may also feel burning, tickling, numbness, and/or prickling sensations in the skin. Polyneuropathy may also cause the patient to experience difficulty breathing. Organomegaly POEMS syndrome often leads to spleen, liver, and/or lymph node enlargement. Endocrinopathy/edema Endocrinopathy occurs when the endocrine glands produce insufficient or excessive levels of their respective hormones. Thus, this symptom of POEMS syndrome depends on the affected gland/hormone. Some potential manifestations of endocrinopathy may include hypogonadism, diabetes mellitus, hypothyroidism, and primary adrenal insufficiency. Gynecomastia or impotence may develop among men, whereas women may experience amenorrhea, enlarged breasts, abnormal lactation, and/or galactorrhea. The ‘E’ in POEMS syndrome can also refer to edematous tissues. This may arise due to increased fluid in the arms and legs, pericardium, peritoneal cavity, or behind the optic nerve. Monoclonal gammopathy Monoclonal gammopathy is the presence of excessive monoclonal (M) proteins in the blood due to the circulation of abnormal plasma cells. In addition to high M protein levels, monoclonal gammopathy may cause plasmacytomas and/or osteosclerosis. Skin changes Several dermatological symptoms are associated with POEMS syndrome, including hyperpigmentation, excessive hair growth, scleroderma, excessive sweating, and clubbed fingernails. Blue discoloration of extremities, white discoloration of the nails, as well as fat wasting at the temples, may also occur in POEMS syndrome. Other symptoms In addition to the hallmark symptoms of POEMS syndrome, several other systemic manifestations of this disorder have been described. Within the blood, for example, POEMS syndrome may lead to thrombocytosis, arterial and/or venous thrombosis, pulmonary arterial hypertension, and increased red blood cell (RBC) counts. Aside from its presence due to polyneuropathy, difficulty breathing may also be caused by fluid within the lungs, respiratory insufficiency, or reduced total lung capacity. Patients with POEMS syndrome may also present with headaches, nausea, weight loss, and fatigue. What is POEMS?Play Pathophysiology Although the exact cause of POEMS syndrome remains unknown, several mechanisms are believed to contribute to the pathophysiology of this disorder. Vascular endothelial growth factor (VEGF), produced by malignant plasma cells, is often present at increased levels in POEMS patients. Elevated VEGF levels can increase the permeability of vascular tissues, as well as lead to angiogenesis and the growth of endothelial cells. Aside from VEGF, other pro-inflammatory cytokines have been implicated in POEMS syndrome, the most notable of which include interleukin 1β (IL-1β), IL-6, and tumor necrosis factor α (TNF-α). These cytokines are often produced at high levels in POEMS syndrome, subsequently increasing inflammation. Diagnosis Both polyneuropathy and monoclonal gammopathy must be present for a POEMS diagnosis. Suspected POEMS syndrome cases should undergo a thorough clinical evaluation consisting of a physical exam, medical history, and laboratory testing. Furthermore, the treating clinician should examine the patient’s eyes, skin, neurological status, and overall organ function. Increased levels of M-proteins, blood plasma, VEGF, and the aforementioned pro-inflammatory cytokines within the serum may be used to support a POEMS syndrome diagnosis. Radiographic imaging may also detect osteosclerotic lesions. Treatment Typically, the treatment for POEMS syndrome is symptomatic. For example, patients with localized osteosclerotic lesions may benefit from radiation treatment and/or surgical removal of the lesion. Conversely, widespread osteosclerotic lesions may be managed with certain antineoplastic agents to improve associated symptoms in these patients. In addition to managing extensive osteosclerosis, chemotherapy may eliminate excessive M proteins circulating in the blood of POEMS syndrome patients. References Faizan, U., Sana, M. K., Farooqi, M. S., & Hasmi, H. (2022). Efficacy and Safety of Regimens Used for the Treatment of POEMS Syndrome – A Systematic Review. Clinical Lymphoma Myeloma and Leukemia 22(2); e26-e33. doi:10.1016/j.clml.2021.07.033. Plasma Cell Neoplasms (Including Multiple Myeloma) Treatment (PDQ®) – Patient Version [Online]. Available from: https://www.cancer.gov/types/myeloma/patient/myeloma-treatment-pdq. POEMS syndrome [Online]. Available from: https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678. POEMS Syndrome [Online]. Available from: https://rarediseases.org/rare-diseases/poems-syndrome/. Jurczyszyn, A., Castillo, J. J., Olszewska-Szopa, M., et al. (2022). POEMS Syndrome: Real World Experience in Diagnosis and Systemic Therapy – 108 Patients Multicenter Analysis. Clinical Lymphoma Myeloma and Leukemia 22(5); 297-304. doi:10.1016/j.clml.2021.10.007. Further reading

Chad Tang, MD, The University of Texas MD Anderson Cancer Center, discusses the unmet needs for patients with oligometastatic prostate cancer. There have been randomized trials for oligometastatic prostate cancer, including STOMP (NCT01558427) and ORIOLE (NCT02680587), which randomized patients to receive radiation in metastatic patients vs observation. These trials showed that radiation delayed the PSA progression that occurs among this patient population, and radiation by itself, delays the progression. According to Tang, other trials have also shown that radiation therapy can combine synergistically with hormone therapy. In earlier trials, upfront hormonal therapy for metastatic disease has also resulted in benefits in overall survival when compared with delaying hormone therapy. Advertisement Then, the EXTEND trial (NCT03599765) recently evaluated patients with oligometastatic prostate cancer and showed there to be improvements in progression-free survival (PFS) and eugonadal PFS when given the combination of metastasis-directed therapy with intermittent hormone therapy. However, Tang notes that questions still remain regarding how experts think about oligometastatic prostate cancer, the way it is defined, and how it is treated. Transcription: 0:10 | I think the biggest unmet need is how we think about oligometastatic prostate cancer. In my opinion, there are 2 ways that you can change therapy with high level evidence. One is to do a randomized control compared with standard of care and number 2 is staging changes. With PSMA PETS and with improved imaging overall, maybe we need to better define, biologically, what oligometastatic prostate cancer is. If that’s true, then maybe we should treat them like metastatic prostate cancers to understand these processes a little bit better and find the optimum treatment recommendations for these patients. REFERENCE: Tang C, Sherry AD, Haymaker C, et al. Addition of metastasis-directed therapy to intermittent hormone therapy for oligometastatic prostate cancer (EXTEND): A multicenter, randomized phase II trial. J Radiat Oncol. 2022;114(5):P1059-1060. doi:10.1016/j.ijrobp.2022.09.006

Prioty Islam, MD, MSc, attending physician, medical oncologist, Leukemia, Division of Hematologic Malignancies, Memorial Sloan Kettering Cancer Center, discusses the exploration of novel roles for BTK inhibitors in the treatment of patients with mantle cell lymphoma (MCL) and chronic lymphocytic leukemia (MCL). Due to recent successes derived from treatment with BTK inhibitors in CLL and MCL, there continues to be interest in furthering their utility by harnessing novel combination regimens, targeting mechanisms, and prognostic features, Islam begins. Advertisement Many clinical trials are evaluating the addition of BTK inhibitors with BCL2 inhibitors, such as an investigation of frontline acalabrutinib (Calquence) and venetoclax (Venclexta), Islam states. This could provide patients with an all-oral, time-limited treatment alternative to either standard continuous BTK inhibitor therapy or the combination of a BCL2 inhibitor and a monoclonal antibody infusion, Islam explains. The goal of further developing novel combination regimens is to improve upon the convenience, tolerability, and efficacy of currently available strategies, she adds. Moreover, there is additional interest in if minimal residual disease (MRD) may inform the duration of BTK inhibitor treatment, Islam continues. Any BTK inhibitors that are currently approved for use are approved with continuous administration until disease progression or intolerance, she says. Some patient subsets may benefit more from a truncated or attenuated treatment course, but this population has not yet been identified, Islam notes. To utilize a time-limited treatment strategy with BTK inhibitors, co-administration of an additional novel therapy may be necessary. This could induce deeper remissions, as complete responses with BTK inhibitor monotherapy are typically uncommon, Islam details. Lastly, an emerging area of research in both MCL and CLL is focused on discovering alternative methods of targeting the BTK protein, Islam says. Current BTK inhibitors are either covalent, in which the agent irreversibly binds to the BTK protein, or noncovalent, in which this bond is reversible. However, there may be other ways to inhibit BTK mutations. For example, several agents currently in development have been designed to degrade the BTK protein, Islam introduces. Because this approach would blockthe BTK signaling pathway in its entirety, this method could also overcome or prevent the development of BTK inhibitor resistance through its varied mechanisms, Islam concludes. Disclosures: Dr Islam reports consulting roles with AbbVie, AstraZeneca, BeiGene, DAVA Oncology, and LOXO Oncology; as well as speaking roles with Targeted Oncology and The Video Journal of Hematologic Oncology (VJHemOnc).

Anna B. Halpern, MD, physician, assistant professor, Clinical Research Division, Fred Hutch; assistant professor, hematology, University of Washington School of Medicine, discusses investigational efforts being developed to expand on the use of ruxolitinib and navitoclax in earlier treatment lines for patients with myelofibrosis. In cohort 3 of the phase 2 REFINE trial (NCT03222609), the combination of ruxolitinib and navitoclax was evaluated in the upfront setting for patients (n=32) who had not been previously exposed to a JAK inhibitor. The study’s primary end point was spleen volume reduction of 35% or greater from baseline at week 24. An exploratory analysis of this cohort was presented at the 2022 ASH Annual Meeting and Exposition, Halpern begins. Findings showed that navitoclax plus ruxolitinib produced a spleen volume reduction of at least 35% at week 24 across specific patient subsets, she details. These subsets consisted of patients 75 years of age or older, those with a high Dynamic International Prognostic Scoring System score, and those with HMR mutations. The percentage of patients who experienced optimal spleen volume reduction in these subgroups are 50%, 33%, and 47%, respectively. Notably, changes in bone marrow fibrosis and reductions in the variant allele frequency (VAF) of the driver gene mutation were seen with the combination regimen in many patients, Halpern continues. Half of patients achieved a greater than 20% reduction in VAF from baseline at week 12 or 24, while a greater than 50% VAF reduction from baseline occurred in 18% of patients. When comparing those with or without HMR mutations, no differences in greater than 20% VAF reduction from baseline to week 12 or 24 were observed between populations. These results indicate the potential disease-modifying ability of ruxolitinib and navitoclax, suggesting that reductions in bone marrow fibrosis and VAF may serve as biomarkers for disease modification, Halpern states. Notably, long-term outcomes cannot be definitively assessed as correlates for leukemia, progression, and survival, she adds. The viability of these 2 biomarker candidates should be assessed more short term, and in larger study populations, Halpern concludes.

Rami Komrokji, MD Although the current classification systems for patients with myelodysplastic syndromes (MDS) were able to identify groups of patients with similar outcomes, further refinement is needed to create more consistent criteria, according to findings from an international dataset analysis of the 2 classification systems presented by Rami Komrokji, MD, during the 2023 EHA Congress. “It’s clear that we are moving to a new era in the classification of MDS and that we have molecularly defined groups; those patients should be thought of, treated, and enrolled in clinical trials separately,” Komrokji said. “Then we have the morphologically defined groups, but we definitely need to step up, try to harmonize those [criteria], and go back into 1 classification that hematopathologists, clinicians, and clinical trialists use as a common language rather than having 2 different classifications.” The analysis, which was conducted on behalf of the International Consortium for MDS, used datasets from Moffitt Cancer Center and the European initiative GenoMed4all:GM encompassing 7017 patients with available molecular data to validate and compare the World Health Organization (WHO) and International Consensus Classification (ICC) 2022 classification criteria. Results showed that patients with deletion 5q (del5q) in the WHO (n = 107) and ICC groups (n = 108) from the Moffitt cohort both achieved an overall survival (OS) of 75.6 months vs 82.1 months for those from GenoMed4all:GM with del5q in the WHO (n = 219) and ICC groups (n = 223), respectively. Patients with SF3B1 mutations, which represent approximately 12% of all MDS cases, had the best outcomes compared with those with del5q and TP53-mutated disease. These patients in the WHO (n = 294) and ICC groups (n = 277) from the Moffitt cohort experienced an OS of 101.8 months and 111.6 months vs 104.9 months and 101.9 months in the GenoMed4all:GM WHO (n = 654) and ICC (n = 594) groups, respectively. In an interview with OncLive®, Komrokji, the vice chair of the Malignant Hematology Department and the head of the Leukemia and MDS Section at Moffitt Cancer Center in Tampa, Florida, discussed the comparative analysis as well as the next steps being taken to develop a more harmonized classification system. OncLive: What was the rationale behind the analysis presented at the 2023 EHA Congress? Komrokji: The classification for MDS has been evolving over the years, from FAB [French American British] in the 1980s, to different iterations from the WHO, but [for] the first time, in 2022 we have 2 classification systems: the WHO and the ICC. Both of them are an attempt to improve the classification for MDS, but also it creates a dilemma in the field and controversy regarding which one to follow. There are some differences [between the classification systems]. It was important to generate data-driven evidence to hopefully guide future harmonization of those 2 classifications. People are mixing classification vs prognostic models. Risk stratification in MDS is based on [the] Revised International Prognostic Scoring System [IPSS-R]. The classification historically is pathological classification [and] it should reflect a unique biology of the disease [as well as] unique groups that have a certain clinical phenotype [where] maybe the pathogenesis of defective hematopoiesis is similar in that group. We were trying to validate those and hopefully this is the first step in trying to come up with harmonization and [return to] 1 classification [system] in MDS. How was the analysis conducted? When the [ICC 2022] classification came out we looked at our database—we have a large database with more than 2500 patients fully annotated—and then we approached our colleagues in Europe. They kindly joined us with the GenoMed4all:GM MDS database, which is the MDS database for almost the whole [of] Europe. Advertisement Together, we were able to put approximately 7000 patients with MDS [into our platform], which is a very large cohort. Hopefully [it] will be a platform not only for this project, but for several other projects down the road. We reclassified all the patients according to the WHO and ICC [criteria], included only patients with fully annotated molecular data, and we looked at the questions that we wanted to address. What were the key findings of the analysis? The 2 classifications recognize molecularly-defined categories of MDS, namely SF3B1, del5q, as well as P53. There are slight differences in the definitions between both of them. For example, we demonstrated that SF3B1 is a unique category, approximately 12% to 13% of patients with MDS have very favorable outcomes with a median OS of more than 10 years [and] low chance of [dying from] leukemia. We also looked at a subset of patients [with] MDS with ring sideroblasts, which is usually the phenotype we see with SF3B1, but [we examined patients] if they were SF3B1 wild-type. That’s a smaller subset, approximately only 4% of patients, but they did not have the same favorable outcomes. The WHO retained [classification of] MDS with ring sideroblasts, which is probably something that could go away down the road in the future based on this. However, we have to think of those models in terms of generalizing them. In countries where there is no access to SF3B1 [testing], ring sideroblasts are still a good marker for SF3B1. We also confirmed that [the patient population with] del5q is a favorable group, [constituting] approximately 5% of patients [with MDS]. Bi-allelic TP53 [is found in] approximately 10% of patients [with MDS] and unfortunately they have very poor outcomes. The ICC proposed a different group of P53, monoallelic. If the blasts were more than 10%, they would consider that MDS/AML with P53. Those patients had worse outcomes and part of it is driven by the increased blast [percentage]. In the future, that category may go away, and we’ll probably stick more to the bi-allelic [characterization of] P53. In the morphologically defined [criteria], we showed that MDS ring sideroblasts without SF3B1 [mutations] are not different. The ICC proposed that the number of lineage dysplasia, whether we have multilineage vs single lineage dysplasia, does matter [and we showed that] multilineage dysplasia did worse.

For patients with myeloproliferative neoplasms (MPNs) — blood cancers that cause the bone marrow to overproduce red or white blood cells or platelets — being able to engage in educated and productive conversations with their care team can be crucial. “There are so many variables in cancer from not only the diagnoses, but ‘how do we treat it?’ to what the prognosis is, and that’s always evolving (so) that it’s hard for providers to keep up with that, let alone patients,” said Charina Toste, a nurse practitioner specializing in oncology and hematology at OptumCare Cancer Care and a professor at Chamberlain College of Nursing, both located in Las Vegas, Nevada. When it comes to the vast category of MPNS (which includes a range of diseases such as myelofibrosis, essential thrombocythemia, and polycythemia vera) patients don’t know what they don’t know. “(Patients) don’t always know, what is the treatment that’s out there? What are the clinical trials that are out there? Oh, and then let’s talk about symptom management, how is my life going to change? How is this going to affect me? How is this going to affect my family? These are questions patients don’t even know to ask. And they trust their health care provider to have the three or four hours it takes to educate them at an appointment that usually is only 15 to 30 minutes.” Toste spoke with CUREⓇ about the importance of education for patients with MPNs in order to empower themselves to have informed conversations with their care team. CUREⓇ: In general, why is it so important for patients to educate themselves, and be prepared to have informed conversations with their care team as they’re going through their cancer journey? Patients with myeloproliferative neoplams should learn about their disease to ensure that they know what questions to ask, a nurse practitioner said. Toste: I think because with any type of diagnosis, there’s kind of the shock factor. And once you get over the shock factor, it’s a different language that patients are learning, it’s a different lifestyle that they’re learning, they have to learn to adjust their entire life for it. And many patients don’t know what to ask because they don’t know what they don’t know. So, I think it’s important to at least have a solid basis of information about your disease, your cancer diagnosis, so you know what to ask. There are so many variables in cancer, from not only the diagnoses, but how we treat it to what the prognosis is, and that’s always evolving (so) that it’s hard for providers to keep up with that, let alone patients. Advertisement What sorts of questions should patients be prioritizing, especially if they are early on in the experience? ‘What is their current diagnoses?’ specifically, so that they understand their diagnoses. Quite honestly, they hear the words and they don’t understand what those words mean; if they see myeloproliferative neoplasms, that’s all they might look up and not know their specific diagnoses (or) that there are different types underneath there. As we well know, there are so many different hematological malignancies. And when you say the word leukemia, there are 200 different types of leukemia. So, you have to know exactly what you have. And what does that mean to you? What does that mean, as far as prognosis? What can I expect now? And what can I expect in the future? So they can adjust their life. Because if anything, after a world pandemic has happened, we realize we can’t always predict the future and we have to enjoy what we have to the best (of our) abilities. So, does this mean I have to quit my job? Does this mean I need to adjust my family lifestyle? Should I move to where I have family and support? Will I be OK here on my own? I think those are the questions that they need to ask: how is it going to impact them personally now and in the future so they can prepare? What are some specific challenges or roadblocks related to MPNs that make it a particularly disease type for patients to inform themselves on? Not always but usually, most of these diagnoses are based in an elderly population. So, with the myelofibrosis and the polycythemia vera, you’re usually looking around the 60s or 70s age group. And for a lot of these patients, they don’t always have the best support, so they don’t always know how to go to Dr. Google and look everything up. They don’t always know what the latest clinical trials are, they don’t always know how to ask those questions. And they aren’t always surrounded by family members, their children are grown, they have their own lives. So, they don’t always have that support that others would have. Sometimes they’re on their own, and they don’t have transportation. They’re wondering about the basics: economics, transportation, those kinds of things. So that’s how it affects them. And those can be some of the obstacles going forward for these patients in obtaining information. And then (for) some of these patients, some are working, some aren’t some are active and family, some aren’t. Some are socially active. And I also think in an elderly population, what are some of the symptoms, when you look at a patient and they go, ‘Well, yes, I’m tired. Yes, I have bone pain, but I’m 80. How do I know that this is disease related?’ So, I think those are also some of the obstacles. Whereas if you’re 20 or 30 years old, and you’re saying, ‘Wow, I have a lot of bone pain, I have a lot of fatigue, I have memory loss,’ that’s going to seem unusual at that age versus if you’re older, a lot of times you just take it as the age and the sands of time moving forward. First, is this actual disease might be progressing. Say there is a patient who is kind

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