Annual Meeting of the ECNMAugust 2020 - Vienna, Austria

Program

EU-US Multicenter Cooperative Initiative to

Standardize Parameters of Disease and Diagnostics forPractice and Clinical Trials in

Mast Cell Disorders

Organization

Peter ValentIrina Sadovnik

Emir HadzijusufovicWolfgang R. Sperr

Venue

Hilton Danube HotelHandelskai 269

1020 ViennaAustria

August 27–29, 2020

E C N MEuropean Competence Network

on Mastocytosis

E C N MEuropean Competence Network

on Mastocytosis

Annual Meeting of the ECNM 2020 Hilton Danube Hotel - Vienna, Austria

Wednesday, August 26, 2020

20:00 Welcome Reception, Hilton Danube Hotel

DAY 1 – Thursday, August 27, 2020

11:00 Start of Registration

12:00 Welcome Coffee

13:00 Welcome Notes and IntroductionPeter Valent (Vienna, Austria)

13:15 Status of the ECNM in 2020 and Future Plan Peter Valent (Vienna, Austria) and Michel Arock (Paris, France)

13:25 Presentation of the Researcher of the Year 2020Massimo Triggiani (Salerno, Italy) and Peter Valent (Vienna, Austria)

13:30 Lecture of the Researcher of the Year 2020Delineation of Patterns of Skin Involvement in Mastocytosis Karin Hartmann (Basel, Switzerland)

14:00 Coffee Break

14:20 Session I: Diagnosis of Systemic MastocytosisChairs: M. Arock & M. Triggiani

14:20 Histopathological Spectrum of Advanced MastocytosisHans-Peter Horny (Munich, Germany)

14:40 Diagnostic Molecular Algorithms in Mastocytosis: Update 2020Gregor Hoermann (Innsbruck, Austria)

15:00 Altered Phenotypic and Functional Immune Profiles in SMAlberto Orfao & Andrea Mayado (Salamanca, Spain)

15:20 Imaging Analyses and Staging in Advanced SM: Status 2020 Andreas Reiter (Mannheim, Germany)

15:40 Coffee Break

16:00 Session II: Prognostication in SM – Status 2020 Chairs: C. Akin & K. Sotlar

16:00 Prognostication in SM: From EARTH to MARS and back to EARTHMohamad Jawhar (Mannheim, Germany)

16:20 International Prognostic Scoring System (IPSM) and Beyond Wolfgang R. Sperr (Vienna, Austria)

16:40 Prognostic Scores for Mastocytosis: which One to Apply and when?Javier I. Muñoz-Gonzalez & Alberto Orfao (Salamanca, Spain)

17:00 Coffee Break

17:20 Session III: Mast Cell Activation, HAT and MCAS Chairs: M. Castells & D.D. Metcalfe

17:20 Definition of MCAS and of Sub-Diagnostic MCA ConditionsPeter Valent (Vienna, Austria)

17:40 Hereditary Alpha-Tryptasemia (HAT): Molecular Mechanisms,Clinical Correlates and Implications for Patient Management Jon Lyons (Bethesda, MD, USA)

18:00 Evaluation of HAT in SM and Clinical ImplicationsGeorg Greiner (Vienna, Austria)

18:20 Session IV: Management and Therapy of MCAS Chairs: H. Oude Elberink & J.H. Butterfield

18:20 Diagnostic and Therapeutic Challenges in MCASCem Akin (Ann Arbor, MI, USA)

18:40 Biomarkers of Mast Cell Activation and MCAS Joseph H. Butterfield (Rochester, MN, USA)

19:00 Summary of DAY 1 and Announcements Peter Valent (Vienna, Austria)

20:00 Dinner at Restaurant Waterfront

DAY 2 – Friday, August 28, 2020

8:00 Welcome Coffee

8:25 Welcome & IntroductionPeter Valent (Vienna, Austria)

8:30 Session V: ECNM Registry ProjectsChairs: W.R. Sperr & P. Valent

8:30 Overview of Projects and Project DiscussionWolfgang R. Sperr (Vienna, Austria)

8:50 Summary and Status of First Wave ProjectsPeter Valent (Vienna, Austria)

9:30 Second Wave Projects

9:30 Characterization and Prognosis in Smoldering SM (PR12)Karin Hartmann (Basel, Switzerland)

9:40 Clinical Impact of Organomegaly in Patients with SM (PR13)Mohamad Jawhar & Andreas Reiter (Mannheim, Germany)

9:50 Impact of Body Mass Index in Patients with Mastocytosis (PR14)Aleksandra Gorska & Marek Niedoszytko (Gdansk, Poland)

10:00 Clinical Features and Course in various Forms of ASM (PR15)Khalid Shoumariyeh & Nikolas von Bubnoff (Freiburg, Germany)

10:10 Clinical Course of Patients with diverse Variants of MCL (PR16)Vanessa Kennedy and Jason Gotlib (Stanford, CA, USA)

10:20 Coffee Break

10:40 Third Wave Projects

10:40 Pure Cutaneous Mastocytosis in Adults (PR18)Knut Brockow (Munich, Germany)

10:50 Sub-types of AHN: Course & Prognostic Features in Variants (PR19)Olivier Hermine (Paris, France)

11:00 Bone Lesions and related Symptoms in Mastocytosis (PR20)Akif Selim Yavuz (Istanbul, Turkey)

11:10 Prognostic Impact of the Percentage of Mast Cells in BM Smears versus BM Histologies (PR21)Vito Sabato (Antwerpen, Belgium)

11:20 Diagnostic and Prognostic Impact of Cytogenetic Abnormalities (PR22)Dominique Bullens & Christine Breynaert (Leuven, Belgium)

11:30 Prognostic Impact of Platelet Counts in SM (PR23)Deborah Christen & Jens Panse (Aachen, Germany)

11:40 Coffee Break

12:00 Fourth-, Fifth- and Sixth Wave Projects

12:00 Pediatric Mastocytosis: Clinical Features and Course in various Forms of the Disease (PR29)Magdalena Lange & Marek Niedoszytko (Gdansk, Poland)

12:10 Prognostic Impact of Monocytes in Mastocytosis (PR30)Friederike Wortmann & Nikolas von Bubnoff (Lübeck, Germany)

12:20 Additional Projects Proposed in Waves 4, 5 and 6Peter Valent (Vienna, Austria)

12:40 Summary of Registry Projects and General Discussion Peter Valent and ECNM Registry Group

12:50 Lunch Break

14:00 Closed ECNM Registry Steering Committee Meeting

15:00 Session VI: Rare Forms of Mastocytosis Chairs: G. Hoermann & B. Nedoszytko

15:00 Familial Mastocytosis: Genetic Background and Clinical SpectrumKarin Hartmann (Basel, Switzerland)

15:20 Diagnosis, Management and Outcome in Mast Cell SarcomaKaroline V. Gleixner (Vienna, Austria)

15:40 Well-Differentiated Mastocytosis: Diagnosis, Course and Prognosis Iván Alvarez-Twose (Toledo, Spain)

16:00 Coffee Break

16:30 Session VII: Cytoreductive and Targeted Therapy in SMChairs: J. Gotlib & O. Hermine

16:30 Therapeutic Algorithms in Advanced SM: Status 2020 Andreas Reiter (Mannheim, Germany)

16:50 Avapritinib in Advanced SM: Update 2020Jason Gotlib (Stanford, CA, USA)

17:20 Comparison of various TKI in ISM and AdvSMCem Akin (Ann Arbor, MI, USA)

17:40 HSCT in AdvSM: Patient-Selection and Preparation Celalettin Ustun (Chicago, IL, USA)

18:00 Side Effect Profiles of TKI in MastocytosisDeepti Radia (London, UK)

18:20 Summary of DAY 2: Peter Valent

19:00 Special Exhibition by Irina Sadovnik (Vienna, Austria) + Grill Buffet

DAY 3 – Saturday, August 29, 2020

8:00 Welcome Coffee

8:20 Welcome & IntroductionPeter Valent (Vienna, Austria)

8:25 Key Note Lectures: chaired and moderated by: Luis Escribano & Mariana Castells

8:30 Key Note Lecture 1: New Insights into the Roles of Mast Cells in Health and DiseaseStephen J. Galli (Stanford, CA, USA)

9:00 Key Note Lecture 2: Mast Cell Signaling Nodes relevant to MCADean D. Metcalfe (Bethesda, MD, USA)

9:30 Session VIII: Pediatric MastocytosisChairs: M. Niedoszytko & S. Broesby-Olsen

9:30 Diagnosis and Prognostication in Pediatric MastocytosisMelody Carter (Bethesda, MD, USA)

9:50 Management of Pediatric Patients with CM and SMMagdalena Lange (Gdansk, Poland)

10:10 Coffee Break

10:40 Session IX: Allergy, MCAS and Mental Issues in SM Chairs: L.B. Schwartz & M. Carter

10:40 IgE-Dependent Allergies and MCAS in SM Marek Niedoszytko (Gdansk, Poland)

11:00 Management of Hymenoptera Venom Allergy in SMHanneke Oude Elberink (Groningen, Netherlands)

11:20 Drug Hypersensitivity: Diagnosis and Management Mariana Castells (Boston, MA, USA)

11:40 Neurological, Psychological and Psychiatric Aspects of SM Olivier Hermine (Paris, France)

12:00 Session X: Diagnosis and Management of AHN Chairs: T. George & H. Kluin-Nelemans

12:00 Spectrum of AHN in SM: Diagnosis and Management Juliana Schwaab (Mannheim, Germany)

12:20 SM-MDS: Diagnosis, Variants and TherapyRoberta Zanotti (Verona, Italy)

12:40 Lunch Break

14:00 Session XI: Networking Activities

14:00 Academic Networking in the USA: AIMCem Akin & Jason Gotlib

14:20 The ECNM: History and PerspectivesPeter Valent & Massimo Triggiani

14:40 Coffee Break

15:30 Session XII: Perspectives and Wishes of PatientsChairs: J. Schwaab & K. Hartmann & F. Siebenhaar

Patients´ Perspectives, Questions and Wishes - MastocytosisThree Patient Group Representatives

Patients´ Perspectives, Questions and Wishes - MCASThree Patient Group Representatives

17:30 General Discussion and Summary: Peter Valent

AB SCIENCE

E C N MEuropean Competence Network

on Mastocytosis

E C N MEuropean Competence Network

on Mastocytosis

AmericanInitiative in

Mast Cell Diseases

Partners and Sponsors

Austrian Competence Network on Mastocytosis

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