E C N MEuropean Competence Network

on Mastocytosis

October 3rd - 5th, 2019Imlauer Hotel PitterSalzburg, Austria

17th Annual Meeting of the

European Competence Network on Mastocytosis (ECNM)

Program

17th Annual Meeting of the European Competence Network on Mastocytosis

OFFICIAL ORGANIZER University Hospital SalzburgParacelsus Medical UniversitySalzburg, Austria

CONFERENCE PRESIDENT Karl Sotlar, The Institute of Pathology, Paracelsus Medical University of Salzburg (PMU), Landeskrankenhaus Salzburg – University Hospital of PMU

LOCAL SCIENTIFIC ADVISORY BOARD Karl Sotlar (Salzburg), Wolfgang Sperr (Vienna), Peter Valent (Vienna)

INTERNATIONAL ADVISORY BOARD Cem Akin (Boston, MA, USA), Michel Arock (Paris), Jason R. Gotlib (Stanford, CA, USA), Karin Hartmann (Bern), Hans-Peter Horny (Munich), Dean D. Metcalfe (Bethesda, MA, USA), Lawrence B. Schwartz (Richmont, VA, USA)

PCO pdl - Dr. Eugen PreußAndreas-Hofer-Str. 6 EG, 6020 Innsbruck, Austria

TARGET AUDIENCE Experts from all disciplines of applied medicine, including clinical and preclinical specialists, as well as natural scientists.

CME – CONTINUOUS MEDICAL EDUCATION We have applied for 19 continuous medical education points(Thursday – 5 points (ID: 660021), Friday – 9 points (ID 661938), Saturday – 5 points (ID 661942))

ECNM NETWORK WEBPAGEwww.ecnm.net

CONFERENCE WEBPAGEwww.ecnm.eu

17th Annual Meeting of the European Competence Network on Mastocytosis

VENUEImlauer Hotel Pitter Salzburg

Rainerstraße 6, 5020 Salzburg, Austriawww.imlauer.com

Lecture HallExhibition Area

Exhibition Area

Registration Desk

Registration Desk

Closed ECNM Registry Steering Committee Meeting

www.ecnm.eu

Thursday, October 3, 2019

12:00 Start of registration13:30 Welcome Coffee14:00 Welcome address

Karl Sotlar and Michael Nake (Salzburg, Austria)14:15 Status of the ECNM in 2019 and Masterplan until 2025

Peter Valent (Vienna, Austria) and Michel Arock (Paris, France)14:25 Presentation of the Researcher of the Year 2019

Michel Arock (Paris, France) and Peter Valent (Vienna, Austria)14:30 Lecture of the Researcher of the Year 2019:

Development of KIT-Targeting Therapy in Advanced MastocytosisJason Gotlib (Stanford, CA, USA)

15:00 Coffee Break15:20 Session 1: Diagnosis of Mastocytosis

Chairs: M. Arock & J. Gotlib15:20 Hematopathology in Mastocytosis: Update 2019

Hans-Peter Horny (Munich, Germany)15:40 The Skin in Mastocytosis: Diagnosis and Therapy

Karin Hartmann (Basel, Switzerland)16:00 Impact of Flow Cytometry and NGS in the Diagnosis of SM

Alberto Orfao (Salamanca, Spain)16:20 Comprehensive Prognostication in Systemic Mastocytosis

Mohamad Jawhar (Mannheim, Germany)16:40 Coffee Break17:00 Session 2: Mast Cell Activation and MCAS

Chairs: M. Castells & K. Hartmann17:00 Mast Cell Activation Syndromes: Facts and Fiction

Peter Valent (Vienna, Austria)17:20 Novel Bone Marrow Findings in Patients with MCAS with Elevated Tryptase:

Diagnostic ImplicationsMariana Castells (Boston, MA, USA)

17:40 Diagnostic and Therapeutic Challenges in MCASCem Akin (Ann Arbor, MI, USA)

18:00 Coffee Break18:20 Session 3: Therapy in SM and MCAS: Status 2019

Chairs: A. Orfao & M. Jawhar18:20 Treatment of Primary and Secondary MCAS in Mastocytosis

Frank Siebenhaar (Berlin, Germany)18:40 Challenges in the Treatment of Advanced SM

Mohamad Jawhar (Mannheim, Germany)19:00 Treatment of Osteopathy in SM

Olivier Hermine (Paris, France)20:00 Welcome Cocktail in the Exhibition Area (ground floor)

followed by Dinner in the Sky Restaurant (top floor)

Friday, October 4, 2019

8:10 Welcome Coffee8:30 Session 4: ECNM Registry Projects

Chairs: W.R. Sperr & P. Valent8:30 Overview of Projects and General Issues

Wolfgang R. Sperr (Vienna, Austria)8:50 Summary and Status of First Wave Projects

Peter Valent (Vienna, Austria)

9:30 Second Wave Projects9:30 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 & Jason Gotlib (Stanford, USA)10:20 Features, Course and Prognosis in Typical ISM (PR17)

Jakub Trizuljak & Michael Doubek (Brno, Czech Republic)10.30 Coffee Break11:00 Third Wave Projects11:00 Pure Cutaneous Mastocytosis in Adults: Clinical Features/Course (PR18)

Julia Winkler, Hans-Peter Horny, Knut Brockow (Munich, Germany)11:10 Sub-types of AHN: Course & Prognostic Features in AHN-Variants (PR19)

Olivier Hermine (Paris, France)11:20 Bone Lesions and related Symptoms in Mastocytosis:

Clinical and Laboratory Correlates and Clinical Significance (PR20)Akif Selim Yavuz (Istanbul, Turkey)

11:30 Prognostic Impact of the Percentage of Mast Cells in BM Smears versus BM Histologies (PR21)

Vito Sabato (Antwerpen, Belgium)11:40 Diagnostic and Prognostic Impact of Cytogenetic Abnormalities (PR22)

Christine Breynaert (Leuven, Belgium)11:50 Prognostic Impact of Platelet Counts in SM and Comparison to Hb and ANC (PR23)

Deborah Christen (Aachen, Germany)12:00 Fourth and Fifth Wave Projects12:00 Clinical Significance of Eosinophilia in Mastocytosis (PR26)

Hanneke Kluin Nelemans (Groningen, Netherlands)12:10 Pediatric Mastocytosis:

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

12:20 Prognostic Impact of Monocytes in MastocytosisFriederike Wortmann & Nikolas von Bubnoff (Lübeck, Germany)

12:30 Summary of Registry Projects and General DiscussionPeter Valent and ECNM Registry Group

12:40 Lunch Break14:00 Closed ECNM Registry Steering Committee Meeting

(Room Markus Sittikus)

15:00 New Centers of the ECNM and other NetworksChairs: Boguslaw Nedoszytko & Hans-Peter Horny

15:00 Experience from the Department of Dermatology and Venereology, I.M. Sechenov First Moscow State Medical University

Polina Pyatilova, Elena Borzova & Olga Olisova (Moscow, Russia)15:20 Overview of the EMBREN Network

Frank Siebenhaar (Berlin Germany)15:40 Experience of the National Research Center for Hematology, Moscow,

in Diagnosis and Management of Adult Patients with Systemic MastocytosisKira Lukina & Alla Kovrigina (Moscow, Russia)

16:00 New Histopathological Markers in SM and Presentation of theSalzburg Center of Excellence of the ECNM

Karl Sotlar (Salzburg, Austria)16:20 Coffee Break

16:40 Session 5: New Diagnostic and Therapeutic ConceptsChairs: S. Broesby-Olsen & M. Triggiani

16:40 Hypertryptasemia and Related DisordersLawrence B. Schwartz (Richmond, VA, USA)

17:00 Management of Pediatric MastocytosisMagdalene Lange (Gdansk, Poland)

17:20 Diagnosis and Therapy of Concomitant Allergies in SMHanneke Oude Elberink (Groningen, Netherland)

17:40 Diagnostic Algorithms in Mast Cell Disorders: Status 2019Michel Arock (Paris, France)

18:00 Use of KIT-targeting TKI in Indolent Systemic MastocytosisCem Akin (Ann Arbor, MI, USA)

20:00 Speakers Dinner

Saturday, October 5, 2019

8:10 Welcome Coffee8:30 Session 6: Management and Therapy of Advanced SM

Chairs: H. Kluin-Nelemans & C. Akin8:30 Mast Cell Sarcoma: Management and Therapeutic Options

Karoline V. Gleixner (Vienna, Austria)8:50 Avapritinib in Systemic Mastocytosis: Update of Ongoing Trials

Jason Gotlib (Stanford, CA, USA)9:10 Therapeutic Options in Mast Cell Leukemia: Status 2019

Andreas Reiter (Mannheim, Germany)9:30 Use of KIT TKI in Various Forms of Advanced SM

Ivan Alvarez-Twose (Toledo, Spain)9:50 Coffee Break

10:20 Session 7: Basic and Translational ScienceChairs: L.B. Schwartz & T. George

10:20 iPSC-Derived Mast Cells in SM: A New Research ToolMartin Zenke (Aachen, Germany)

10:40 Niche-Mediated Resistance of Neoplastic Mast CellsGregor Eisenwort (Vienna, Austria)

11:00 Comparison of MARS, IPSM, Mayo, and REMA Scores in SMJavier I. Muñoz-Gonzalez & Alberto Orfao (Salamanca, Spain)

11:20 Session 8: Late Breaking AbstractsChairs: K. Sotlar & O. Hermine

11:20 Histopathological Criteria for Mastocytosis in the SkinJulia Winkler, Hans-Peter Horny, Thomas Kristensen & Knut Brockow(Munich, Germany & Odense, Denmark)

11:35 Bone Marrow Response to AvapritinibTracy George (Salt Lake City, UT, USA)

11:50 KIT Mutation Analysis in Clinical Practice – the MastOUH ExperienceThomas K. Kristensen (Odense, Denmark)

12:05 Role of Cytokines in the Pathogenesis of SMGregor Hörmann (Innsbruck & Vienna, Austria)

12:20 Quantification of QoL in Mastocytosis: Status 2019Sigurd Broesby-Olsen (Odense, Denmark)

12:35 CDK4/CDK6 Inhibition: a Potent Approach to block Growth of NeoplasticMast Cells and Synergism with KIT-targeting TKI

Mathias Schneeweiss-Gleixner (Vienna, Austria)12:50 Closing Remarks and Announcements

Karl Sotlar (Salzburg, Austria) and Peter Valent (Vienna, Austria)13:10 End of Meeting

www.ecnm.eu