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    Faculty & Disclosures
    Prof. Dr Vito Sabato

    University of Antwerp, Antwerp, Belgium

    Vito Sabato is a senior staff member of the Department of Immunology–Allergology–Rheumatology at Antwerp University Hospital and is associate professor at the University of Antwerp. read more

    Prof. Sabato was born in Potenza, Italy. After completing his medical studies and training in allergology and clinical immunology at the Catholic University of Rome, Italy, he joined the Antwerp University Hospital, in Belgium.

    His primary clinical and research interests include anaphylaxis, drug hypersensitivity, autoinflammatory disorders and primary mast cell disorders.

    He is the coordinator of the local Mastocytosis Center of Excellence, which is part of the European Competence Network on Mastocytosis.
    He is a senior clinical investigator of the Flemish Research Council and acts as a principal investigator of several clinical trials on mastocytosis.

    Prof. Dr Vito Sabato discloses: Advisory board/panel fees from Blueprint Medicines, Cogent Biosciences, Novartis, Telios Pharma, Inc. Grants/research support from Blueprint Medicines, Cogent Biosciences. Speaker’s bureau fees from Blueprint Medicines.
    Dr Sigurd Broesby-Olsen

    Odense University Hospital, Odense, Denmark

    Sigurd Broesby-Olsen is a consultant dermatologist and head of the Department of Dermatology and Allergy at the Odense University Hospital, and associate professor at the University of Southern Denmark. read more

    He is head of the National Danish Mastocytosis Centre at the Odense University Hospital (MastOUH). This is a multidisciplinary centre caring for paediatric and adult patients with mastocytosis.

    His areas of scientific focus include anaphylaxis and other clinical aspects and comorbidities associated with mastocytosis; the development of sensitive KITD816V mutation analysis in peripheral blood; the establishment of clinical diagnostic algorithms; and guidelines for multidisciplinary management of mastocytosis patients.

    Dr Sigurd Broesby-Olsen has no relevant financial relationships to disclose in relation to this activity.
    Prof. Prithviraj Bose

    MD Anderson Cancer Center University of Texas, TX, USA

    Prithviraj Bose is a professor in the Department of Leukemia at the MD Anderson Cancer Center (MDACC) in Houston, Texas, USA, where he has been in post since 2015. read more

    After obtaining his medical degree from the University of Calcutta in Kolkata, India, where he attended the Calcutta Medical College, Prof. Bose completed an internship in internal medicine at a University of Washington affiliate in Spokane, Washington, and a residency at the Henry Ford Hospital in Detroit, Michigan. This was followed by a fellowship in haematology and oncology at the University of Oklahoma Health Sciences Center in Oklahoma City. He then went on to join the haematology/oncology faculty at Virginia Commonwealth University (VCU) in Richmond, Virginia, where he was an assistant professor of medicine and a member of the National Cancer Institute-designated VCU Massey Cancer Center.

    Prof. Bose’s major clinical and research focus is on myeloproliferative neoplasms (MPNs). He has authored numerous articles on MPN in peer-reviewed journals and books and served on the MPN panel of the National Comprehensive Cancer Network for several years. Prof. Bose is also heavily involved in clinical trial designs for patients with MPN, and also in other areas of MPN research. He is leading a number of important therapeutic clinical trials in myelofibrosis, polycythemia vera and systemic mastocytosis at MDACC. He speaks regularly on MPN at regional, national and international meetings, and co-chairs the annual US Focus meeting on MPNs and myelodysplastic syndromes.

    Prof. Bose has published well over 400 peer-reviewed journal articles, book chapters and scientific meeting abstracts. He regularly reviews articles for numerous haematology journals, grant applications and abstracts for major international conferences, including the American Society of Hematology and European Hematology Association congresses. He serves as an associate editor for Clinical Lymphoma, Myeloma and Leukemia and as the MPN section editor for Current Hematologic Malignancy Reports.

    Prof. Prithviraj Bose discloses: Advisory board/panel fees from Blueprint Medicines, Cogent Biosciences, CTI BioPharma, Incyte, Novartis. Consultancy fees from AbbVie, BMS, Disc Medicine, Geron, GSK, lonis Pharmaceuticals, Jubilant Pharma, Karyopharm Therapeutics, Keros Therapeutics, Morphic Therapeutic, MorphoSys, PharmaEssentia and Sumitomo Pharma.
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    Tutorial

    This icon indicates there is a poll question. Click it when you see it to interact with your peers.

    Poll

    How important do you think the multidisciplinary team is in managing patients with SM?

    Submit your answer to see the results

    Extremely important
       
    Important
       
    Not very important
       
    Not important
       

    Tutorial

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    Poll

    Which emerging treatment approach is likely to have the most impact on ISM management?

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    Next-generation TKIs
       
    Siglec 8 mAbs
       
    BTK inhibitors
       
    Other
       

    Tutorial

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    Poll

    How confident are you in treating patients with SM?

    Submit your answer to see the results

    Not at all
       
    A little
       
    Moderately
       
    Extremely
       

    Tutorial

    This icon indicates there is a poll question. Click it when you see it to interact with your peers.

    Poll

    What is your main challenge in identifying patients with SM?

    Submit your answer to see the results

    Variable presenting symptoms
       
    Lack of awareness of SM
       
    Poor understanding of diagnostic criteria
       
    Challenges in differential diagnosis
       
     
    Videos Take CE/CME Test
    Haematology, Haematological Malignancies CE/CME accredited

    touchPANEL DISCUSSION
    A visually engaging discussion designed to emulate a ‘live’ panel experience and provide clinicians with practical expert insights to address their clinical challenges. Useful tips below will show how to navigate the activity. Close

    Multidisciplinary insights: Navigating the challenges of systemic mastocytosis diagnosis and management

    Faculty Podcast Featured
    Useful tips
    • Select in the video player controls bar to choose subtitle language. Subtitles available in English, French, German, Spanish.
    • A practice aid is available for this activity in the Toolkit
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    Learning Objectives

    After watching this activity, participants should be better able to

    • Describe the signs, symptoms and diagnostic pathway for systemic mastocytosis
    • Explain how to apply personalized treatment strategies using a multidisciplinary approach to effectively manage systemic mastocytosis
    • Recall the latest data for new and emerging treatments for systemic mastocytosis and evaluate their potential impact on the clinical management of systemic mastocytosis in the future
    Overview

    In this activity, an allergist, dermatologist and haemato-oncologist discuss the diagnosis and management of systemic mastocytosis, including current and emerging treatment options and multidisciplinary approaches to optimize patient care.

    This activity is jointly provided by USF Health and touchIME. read more

    Target Audience

    This activity has been designed to meet the educational needs of allergists/immunologists, dermatologists, haematologists, oncologists and primary care providers involved in the management of patients with systemic mastocytosis.

    Disclosures

    USF Health adheres to the Standards for Integrity and Independence in Accredited Continuing Education. All individuals in a position to influence content have disclosed to USF Health any financial relationship with an ineligible organization. USF Health has reviewed and mitigated all relevant financial relationships related to the content of the activity. The relevant relationships are listed below. All individuals not listed have no relevant financial relationships.

    Faculty

    Prof. Dr Vito Sabato discloses: Advisory board/panel fees from Blueprint Medicines, Cogent Biosciences, Novartis, Telios Pharma, Inc. Grants/research support from Blueprint Medicines, Cogent Biosciences. Speaker’s bureau fees from Blueprint Medicines.

    Dr Sigurd Broesby-Olsen has no relevant financial relationships to disclose in relation to this activity.

    Prof. Prithviraj Bose discloses: Advisory board/panel fees from Blueprint Medicines, Cogent Biosciences, CTI BioPharma, Incyte, Novartis. Consultancy fees from AbbVie, BMS, Disc Medicine, Geron, GSK, lonis Pharmaceuticals, Jubilant Pharma, Karyopharm Therapeutics, Keros Therapeutics, Morphic Therapeutic, MorphoSys, PharmaEssentia and Sumitomo Pharma. Grants/research support from Blueprint Medicines, BMS, Cogent Biosciences, Disc Medicine, Geron, lncyte, Ionis Pharmaceuticals, Janssen, Kartos Therapeutics, Karyopharm Therapeutics, MorphoSys and Sumitomo Pharma; CTI BioPharma/Sobi and Telios Pharma (relationships terminated).

    Content reviewer

    Carolina Leon BSN, MSN, ARNP-BC has no financial interests/relationships or affiliations in relation to this activity.

    Touch Medical Director

    Kathy Day has no financial interests/relationships or affiliations in relation to this activity.

    USF Health Office of Continuing Professional Development and touchIME staff have no financial interests/relationships or affiliations in relation to this activity.

    Requirements for Successful Completion

    In order to receive credit for this activity, participants must review the content and complete the post-test and evaluation form. Statements of credit are awarded upon successful completion of the post-test and evaluation form.

    If you have questions regarding credit please contact cpdsupport@usf.edu.

    Accreditations

    Physicians

    This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education (ACCME) through a joint providership of USF Health and touchIME. USF Health is accredited by the ACCME to provide continuing medical education for physicians.

    USF Health designates this enduring material for a maximum of 1.0 AMA PRA Category 1 CreditTM.  Physicians should claim only the credit commensurate with the extent of their participation in the activity.

    The European Union of Medical Specialists (UEMS) – European Accreditation Council for Continuing Medical Education (EACCME) has an agreement of mutual recognition of continuing medical education (CME) credit with the American Medical Association (AMA). European physicians interested in converting AMA PRA Category 1 CreditTM into European CME credit (ECMEC) should contact the UEMS (www.uems.eu).

    Advanced Practice Providers

    Physician Assistants may claim a maximum of 1.0 Category 1 credits for completing this activity. NCCPA accepts AMA PRA Category 1 CreditTM from organizations accredited by ACCME or a recognized state medical society.

    The AANPCP accepts certificates of participation for educational activities approved for AMA PRA Category 1 CreditTM by ACCME-accredited providers. APRNs who participate will receive a certificate of completion commensurate with the extent of their participation.

    Date of original release: 24 July 2024. Date credits expire: 24 July 2025.

    If you have any questions regarding credit please contact cpdsupport@usf.edu.

    This activity is CE/CME accredited

    To obtain the CE/CME credit(s) from this activity, please complete this post-activity test.

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    This activity is funded by:

    An independent medical education grant from Blueprint Medicines. This activity is jointly provided by USF Health and touchIME.

    The information provided by this CME activity is for continuing education purposes only and is not meant to substitute for the independent medical/clinical judgment of a healthcare provider relative to diagnostic and treatment options of a specific patient’s medical condition.

    USF is an Equal Opportunity / Affirmative Action / Equal Access Institution.

    USF Health is accredited by the Accreditation Council for Continuing Medical Education, the American Nurses Credentialing Center and the Accreditation Council for Pharmacy Education to provide continuing education to healthcare professionals. As an accredited provider, USF Health is required to disclose personal information to relevant accredited bodies that certify CME to process credits/contact hours, comply with reporting requirements, and for internal recordkeeping and regulatory purposes. USF Health does not share or sell any individual’s contact information or unique identifiers to any commercial supporter, advertiser, or third party without the specific permission of the individual.

    Unapproved products or unapproved uses of approved products may be discussed by the faculty; these situations may reflect the approval status in one or more jurisdictions. The presenting faculty have been advised by touchIME and USF Health to ensure that they disclose any such references made to unlabelled or unapproved use. No endorsement by touchIME or USF Health of any unapproved products or unapproved uses is either made or implied by mention of these products or uses in touchIME or USF Health activities. touchIME and USF Health accept no responsibility for errors or omissions.

    This content is intended for healthcare professionals.

    Date of original release: Date of original release: 24 July 2024.  Date credits expire: 24 July 2025.

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    touchPANEL DISCUSSION
    Multidisciplinary insights: Navigating the challenges of systemic mastocytosis diagnosis and management
    1.0 CE/CME credit
    Question 1/6
    You are discussing the initial diagnostic work-up of mastocytosis with a junior colleague in your clinic. How might you advise them on the distinguishing features associated with the presentation of ISM vs ASM?

    ASM, advanced SM; GI, gastrointestinal; ISM, indolent SM; SM, systemic mastocytosis.

    The signs and symptoms of SM are diverse. UP/maculopapular skin lesions are a prominent clinical feature of mastocytosis,1 and can be found in patients with CM and ISM; they may also occur in patients with ASM.1,2 GI symptoms, including nausea, vomiting, abdominal pain and diarrhoea are commonly associated with both non-advanced forms (e.g. ISM) and ASM. Many patients with SM experience episodes of unexplained anaphylaxis.1

    Abbreviations
    ASM, advanced SM; CM, cutaneous mastocytosis; GI, gastrointestinal; ISM, indolent SM; SM, systemic mastocytosis; UP, urticaria pigmentosa.

    References

    1. Lee HJ. Blood Res. 2023;58:S96–108.
    2. Hartmann K, et al. J Allergy Clin Immunol. 2016;137:35–45.
    Question 2/6
    Your patient is a 29-year-old woman meeting the ICC/WHO diagnostic criteria for SM. During MDT discussions, which of the following evaluations would you use to distinguish between non-advanced subtypes?

    BM, bone marrow; ICC, ICC, International Consensus Criteria; MC, mast cell; MDT, multidisciplinary team; SM, systemic mastocytosis; WHO, World Health Organization.

    Current ICC/WHO guidelines outline the role of B and C findings in the classification of SM subtypes.1–3 The presence of C findings support the diagnosis of ASM, while evaluation of B findings can distinguish between SSM and ISM, and help identify BMM. The presence of two or more B findings in the absence of C findings suggests a diagnosis of SSM.1,2

    Abbreviations
    ASM, advanced SM; BMM, bone marrow mastocytosis; ICC, International Consensus Criteria; ISM, indolent SM; SM, systemic mastocytosis; SSM, smoldering SM; WHO, World Health Organization.

    References

    1. Veitch S, Radia DH. Hematology Am Soc Hematol Educ Program. 2023:2023:396–406.
    2. Arber DA. et al. Blood. 2022;140:1200–28.
    3. Khoury JD, et al. Leukemia. 2022;36:1703–19.
    Question 3/6
    During a discussion with your 23-year-old patient about their diagnosis of ISM, they express concerns about the potential risks of living with the condition and what it means for their future. As well as prescribing an adrenaline auto-injector, which of the following might you discuss with your patient to support their initial management?

    ISM, indolent SM; SM, systemic mastocytosis.

    As a complex, multisystem disease with a varied clinical course, SM requires a multidisciplinary approach.1 Patients with SM may need psychological or psychiatric support to adjust to their diagnosis.1 Real-world analysis of US healthcare records of patients with SM (N=8,710) showed that 3.9% of patients with ISM (n=7,120) included in the analysis progressed to advanced disease over the 24 month timeframe evaluated.2

    Abbreviations
    ISM, indolent SM; SM, systemic mastocytosis.

    References

    1. Zanotti R, et al. Mediterr J Hematol Infect Dis. 2021;13:e2021068.
    2. Mukherjee S, et al. Presented at: 64th ASH Annual Meeting and Exposition, New Orleans, LA, USA. 10–13 December 2022. Poster 3053.
    Question 4/6
    You are considering treatment options with your 34-year-old patient with ASM. Based on current prescribing guidance surrounding known side-effect risks with approved tyrosine kinase inhibitors, which of the following test results would not support initiation of avapritinib in this patient?

    ASM, advanced systemic mastocytosis.

    Warnings and precautions for avapritinib outlined in prescribing guidance include intracranial haemorrhage and cognitive effects; therefore, in patients with ASM, a platelet count must be performed prior to initiating avapritinib.1,2 Avapritinib is not recommended for the treatment of patients with ASM with platelet counts <50 x 109/L;1,2 in the United States this limitation of use also extends to patients with ISM.2

    Abbreviations
    ASM, advanced SM; ISM, indolent SM; SM, systemic mastocytosis.

    References

    1. EMA. Avapritinib SmPC. Available at: https://rb.gy/m01got (accessed 19 June 2024).
    2. FDA. Avapritinib PI. Available at: https://shorturl.at/NyF2T (accessed 19 June 2024)
    Question 5/6
    According to available real-world data published in 2022 surveying allergists/immunologists and haemato-oncologists with expertise in SM, which of the following statements best reflects treatment goal priorities from an HCP perspective when individualizing SM treatment?

    ASM, advanced SM; HCP, healthcare provider; ISM, indolent SM; SM, systemic mastocytosis.

    A 51-item survey of 111 HCPs involved in the management of patients with SM – including allergy/immunology specialists (n=57) and haemato-oncology specialists (n=54) – with an average of 14 years’ clinical experience, rated the importance of treatment goals in SM, including differences between the ISM and ASM subtypes. Among HCPs surveyed, the two most important treatment goals identified differed between ISM and ASM. In ISM, better quality of life and improvement of symptoms were the two most important treatment goals; in ASM, improved survival outcomes (OS/PFS) and reduced organ damage were rated as the two most important.

    Abbreviations
    ASM, advanced SM; HCP, healthcare provider; ISM, indolent SM; OS, overall survival; PFS, progression-free survival; SM, systemic mastocytosis.

    Reference
    Mesa RA, et al. Cancer. 2022;128:3700–8.

    Question 6/6
    Your patient is a 31-year-old female with ISM who continues to suffer with very severe symptoms despite long-term use of at least two prior H1/H2 inhibitors and mast cell stabilizers. She explains the negative impact that symptoms have on her ability to continue with normal activities. You optimize antimediator treatment, including an up-dosed second generation, non-sedating H1 inhibitor. You also consider off-label omalizumab. Based on currently available EMA-/FDA-approved therapies (and assuming access), which of the following might you consider next in the management of this patient to improve their quality of life if symptoms remain severe and unmanageable?

    EMA, European Medicines Agency; FDA, US Food and Drug Administration; H, histamine; HSCT, haematopoietic stem cell transplantation; ISM, indolent systemic mastocytosis.

    Based on the results of the phase II PIONEER trial (NCT03731260) in patients with ISM,1 avapritinib is currently approved for the treatment of ISM in adults (US), and patients with moderate-to-severe symptoms inadequately controlled on symptomatic treatment (Europe).2,3 Avapritinib was superior to placebo in reducing uncontrolled symptoms and mast cell burden in patients with ISM. From baseline to week 24, avapritinib yielded a decrease of 15.6 points (95% CI, –18.6 to –12.6) in total symptom score vs 9.2 points (95% CI, –13.1 to –5.2) with placebo (p=0.003).1 Midostaurin is currently approved for the treatment of aggressive SM, SM-AHM/AHN and MCL only.4,5

    Abbreviations
    CI, confidence interval; ISM, indolent SM; MCL, mast cell leukaemia; SM, systemic mastocytosis; SM-AHM/AHN, SM with associated haematological malignancy/neoplasm.

    References

    1. Gotlib J, et al. NEJM Evid. 2023;2:EVIDoa2200339.
    2. EMA. Avapritinib SmPC. Available at: https://rb.gy/m01got (accessed 19 June 2024).
    3. FDA. Avapritinib PI. Available at: https://shorturl.at/NyF2T (accessed 19 June 2024)
    4. EMA. Midostaurin. SmPC. Available at: https://rb.gy/vzbjkx (accessed 19 June 2024).
    5. FDA. Midostaurin PI. Available at: https://rb.gy/6k1igk (accessed 19 June 2024)
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    This activity is funded by:

    An independent medical education grant from Blueprint Medicines. This activity is jointly provided by USF Health and touchIME.

    The information provided by this CME activity is for continuing education purposes only and is not meant to substitute for the independent medical/clinical judgment of a healthcare provider relative to diagnostic and treatment options of a specific patient’s medical condition.

    USF is an Equal Opportunity / Affirmative Action / Equal Access Institution.

    USF Health is accredited by the Accreditation Council for Continuing Medical Education, the American Nurses Credentialing Center and the Accreditation Council for Pharmacy Education to provide continuing education to healthcare professionals. As an accredited provider, USF Health is required to disclose personal information to relevant accredited bodies that certify CME to process credits/contact hours, comply with reporting requirements, and for internal recordkeeping and regulatory purposes. USF Health does not share or sell any individual’s contact information or unique identifiers to any commercial supporter, advertiser, or third party without the specific permission of the individual.

    Unapproved products or unapproved uses of approved products may be discussed by the faculty; these situations may reflect the approval status in one or more jurisdictions. The presenting faculty have been advised by touchIME and USF Health to ensure that they disclose any such references made to unlabelled or unapproved use. No endorsement by touchIME or USF Health of any unapproved products or unapproved uses is either made or implied by mention of these products or uses in touchIME or USF Health activities. touchIME and USF Health accept no responsibility for errors or omissions.

    This content is intended for healthcare professionals.

    Date of original release: Date of original release: 24 July 2024.  Date credits expire: 24 July 2025.

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