Evidence map›Paper›PMID 42363232›Full record

ArticleJournal of hematology & oncology2026

Outcomes and prognostic factors in patients with systemic mastocytosis and an associated myeloid neoplasm: an international ECNM registry study.

Olivier Hermine, Orianne Debeaupuis, Gandhi Damaj, Johannes Lübke, Juliana Schwaab, Marc Heizmann, Frank Siebenhaar, Polina Pyatilova, Nicole Nojarov, Marek Niedoszytko and 58 more

Abstract readMulticenter Study
In one paragraph

Article in Journal of hematology & oncology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

68 authors.

Olivier HermineCEREMAST, INSERM U1163, Imagine Institute, Necker-Enfants Malades and Cochin Hospitals, AP-HP, Université Paris Cité, Paris, France. ohermine@gmail.com.
Orianne DebeaupuisCEREMAST, INSERM U1163, Imagine Institute, Necker-Enfants Malades and Cochin Hospitals, AP-HP, Université Paris Cité, Paris, France.
Gandhi DamajCEREMAST, Hematology Institute, Normandy University School of Medicine, Caen, France.
Johannes LübkeDepartment of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany.
Juliana SchwaabDepartment of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany.
Marc HeizmannDivision of Oncology, Hematology and Transfusion Medicine, Kantonsspital Aarau, Switzerland.
Frank SiebenhaarInstitute of Allergology, Charité - Universitätsmedizin Berlin, Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
Polina PyatilovaInstitute of Allergology, Charité - Universitätsmedizin Berlin, Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
Nicole NojarovInstitute of Allergology, Charité - Universitätsmedizin Berlin, Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
Marek NiedoszytkoDepartment of Allergology, Medical University of Gdansk, University Clinical Center Gdansk, Gdańsk, Poland.
Aleksandra GorskaDepartment of Allergology, Medical University of Gdansk, University Clinical Center Gdansk, Gdańsk, Poland.
Mattias MattssonDepartment of Hematology, Uppsala University Hospital and Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden.
Khalid ShoumariyehDepartment of Medicine I, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Stephan de BraDepartment of Medicine I, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Friederike WortmannDepartment of Hematology and Oncology, University Medical Center Schleswig-Holstein (UKSH), University Cancer Center Schleswig-Holstein (UCCSH), Campus Luebeck and University of Lübeck, Lübeck, Germany.
Nikolas von BubnoffDepartment of Hematology and Oncology, University Medical Center Schleswig-Holstein (UKSH), University Cancer Center Schleswig-Holstein (UCCSH), Campus Luebeck and University of Lübeck, Lübeck, Germany.
Horia BumbeaDepartment of Hematology and Bone Marrow Transplant, Emergency University Hospital of Bucharest (SUUB); "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.
Delia SoareDepartment of Hematology and Bone Marrow Transplant, Emergency University Hospital of Bucharest (SUUB); "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.
Chiara ElenaPoliclinico San Matteo Pavia Fondazione IRCCS, Pavia, Italy.
Judit VárkonyiDepartment of Hematology and Internal Medicine, Semmelweis University, Budapest, Hungary.
Anna Belloni FortinaPediatric Dermatology, Internal Medicine, Azienda Ospedaliera, Università di Padova, Padova, Italy.
Simge ErdemDepartment of Internal Medicine, Division of Hematology, Istanbul University Istanbul Faculty of Medicine, Istanbul, Türkiye.
Knut BrockowDepartment of Dermatology and Allergy Biederstein, School of Medicine and Health, Technical University of Munich, Munich, Germany.
Tobias Michael FranzDepartment of Dermatology and Allergy Biederstein, School of Medicine and Health, Technical University of Munich, Munich, Germany.
Jens PanseCenter for Integrated Oncology Aachen Bonn Cologne Düsseldorf (CIO ABCD), Aachen, Germany.
Deborah ChristenCenter for Integrated Oncology Aachen Bonn Cologne Düsseldorf (CIO ABCD), Aachen, Germany.
Christine Breynaert19KU Leuven Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group and MASTeL, UZ Leuven, Leuven, Belgium.
Akif Selim YavuzDivision of Hematology, Istanbul Medical School, University of Istanbul, Istanbul, Turkey.
Luca MalcovatiPoliclinico San Matteo Pavia Fondazione IRCCS, Pavia, Italy.
Michael DoubekDepartment of Internal Medicine - Hematology and Oncology, University Hospital and Masaryk University, Brno, Czech Republic.
Vito SabatoDepartment of Immunology, Allergology Rheumatology University of Antwerp and Antwerp University Hospital, Edegem, Belgium.
Ilaria TanasiHematology Unit, Department of Engineering for Innovation Medicine, Azienda Ospedaliera Universitaria Integrata di Verona, Verona, Italy.
Massimiliano BonifacioHematology Unit, Department of Engineering for Innovation Medicine, Azienda Ospedaliera Universitaria Integrata di Verona, Verona, Italy.
Karin HartmannDivision of Allergy, Departments of Dermatology and Biomedicine, University Hospital Basel and University of Basel, Basel, Switzerland.
Axel RüferDepartment of Hematology, Luzerner Kantonsspital, University of Luzern, Luzern, Switzerland.
Mariarita SciumèS.C. Ematologia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Valentina BellaniS.C. Ematologia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Irena Angelova-FischerKepler University Hospital, Linz, Austria.
Alex StefanKepler University Hospital, Linz, Austria.
Massimo TriggianiDivision of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
Roberta ParenteDivision of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
Saskia K KleinDepartment of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Michael MakrisNational Center of Expertise for Mastocytosis and Mast Cell Disorders, 2nd Dpt. of Dermatology and Venereology, National and Kapodistrian University of Athens, University General Hospital 'Attikon', Athens, Greece.
Sotirios PapageorgiouNational Center of Expertise for Mastocytosis and Mast Cell Disorders, 2nd Dpt. of Dermatology and Venereology, National and Kapodistrian University of Athens, University General Hospital 'Attikon', Athens, Greece.
Paul van DaeleDepartment of internal medicine and department of immunology, Erasmus MC, Rotterdam, The Netherlands.
Ingunn DybedalDepartments of Hematology and Pharmacology, Oslo University Hospital, Oslo, 0372, Norway.
Daniel BaffoeDepartments of Hematology and Pharmacology, Oslo University Hospital, Oslo, 0372, Norway.
Theo GülenDepartment of Respiratory Medicine and Allergy, Karolinska University Hospital Huddinge, Stockholm, Sweden.
Madlen JentzschDepartment of Hematology, Cell Therapy, Hemostaseology and Infectious Diseases, University Hospital Leipzig, Leipzig, Germany.
Olivier LortholaryCEREMAST, INSERM U1163, Imagine Institute, Necker-Enfants Malades and Cochin Hospitals, AP-HP, Université Paris Cité, Paris, France.
Mael HeibligCEREMAST, Hematology Department, Centre Hospitalier Lyon Sud, Pierre-Bénite, France.
Cristina Bulai LivideanuCEREMAST, Dermatology Department, CHU de Toulouse, Toulouse, France.
Stéphane BareteCEREMAST, Sorbonne Université, Unité Fonctionnelle de Dermatologie, Groupe Hospitalier Pitié-Salpêtrière-C. Foix, AP-HP, Paris, France.
Clément GourguechonCEREMAST, Clinical Hematology Department, Amiens-Picardie University Hospital, Amiens, France.
Sophie Dimicoli-SalazarService d'Hématologie Clinique et thérapie cellulaire, CHU de Bordeaux, Pessac, France.
Olivier TournilhacCEREMAST, Clinical Hematology and Cell Therapy Department, Clermont-Ferrand University Hospital, UCA, EA7453 Chelter CIC-1405, Clermont-Ferrand, France.
Laurent FrenzelCEREMAST, INSERM U1163, Imagine Institute, Necker-Enfants Malades and Cochin Hospitals, AP-HP, Université Paris Cité, Paris, France.
Michel ArockCEREMAST, Pitié-Salpêtrière hospital, AP-HP, Sorbonne University, Paris, France.
Andrzej MitalDepartment of Hematology, Medical University of Gdansk, University Clinical Center Gdansk, Gdańsk, Poland.
Johannes N WoltscheDepartment of Dermatology and Venereology, Medical University of Graz, Graz, Austria.
Hanneke C Kluin-NelemansUniversity Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Joanna N G Oude ElberinkDepartment of Allergology, University Medical Center Groningen and GRIAC Research Institute, University of Groningen, Groningen, The Netherlands.
Jason GotlibStanford Cancer Institute/Stanford University School of Medicine, Stanford, CA, USA.
Martina SperrDepartment of Internal Medicine I, Division of Hematology & Hemostaseology, and Medical University of Vienna, Vienna, Austria.
Andreas ReiterDepartment of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany.
Peter ValentDepartment of Internal Medicine I, Division of Hematology & Hemostaseology, and Medical University of Vienna, Vienna, Austria.
Wolfgang R SperrDepartment of Internal Medicine I, Division of Hematology & Hemostaseology, and Medical University of Vienna, Vienna, Austria.
Julien RossignolCEREMAST, INSERM U1163, Imagine Institute, Necker-Enfants Malades and Cochin Hospitals, AP-HP, Université Paris Cité, Paris, France. Julien.rossignol@aphp.fr.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundSystemic mastocytosis (SM) is a spectrum of hematologic disorders characterized by accumulation of atypical mast cells (MCs) in extracutaneous organs. SM with an associated hematologic neoplasm (SM-AHN), the most frequent subtype of advanced SM, is predominantly associated with myeloid neoplasms, consistent with shared clonal architecture. Because of its rarity and heterogeneity, robust outcome data aligned with contemporary classifications are needed to inform risk stratification.

methodsWe analyzed the 10th data wave of the European Competence Network on Mastocytosis registry (34 European centers and 1 US center). SM and AHN diagnoses followed the 2022 World Health Organization classification. Baseline characteristics and overall survival (OS) were compared between patients with myeloid SM-AHN and SM without AHN (SM-no-AHN). Within SM-AHN, outcomes were analyzed by SM component (advanced: aggressive SM [ASM] or MC leukemia [MCL] vs. non-advanced: bone marrow mastocytosis, indolent SM, or smoldering SM) and AHN subtype.

resultsAmong 3,925 patients with SM, 467 (11.9%) had myeloid SM-AHN. Myelodysplastic/myeloproliferative neoplasms (MDS/MPN) were the most frequent AHN category (41.1%), with chronic myelomonocytic leukemia as the most common subtype (29.6%). Compared with SM-no-AHN, SM-AHN patients were older, more often male, and less frequently had skin involvement. The SM component was advanced in 55.0% of SM-AHN versus 5.8% of SM-no-AHN (p < 0.001). OS was shorter in SM-AHN than SM-no-AHN (median 36.1 vs. 340.9 months; p < 0.001) and was reduced across SM subtypes, including ASM (31.0 vs. 81.1 months) and MCL (7.1 vs. 23.2 months). Within SM-AHN, advanced SM-AHN had shorter OS than non-advanced SM-AHN (28.3 vs. 70.9 months; p < 0.001). Median OS differed by AHN subtype (57.0 months in SM-MPN, 35.7 in SM-MDS, 34.2 in SM-MDS/MPN, and 14.7 in SM associated with acute myeloid leukemia; p < 0.001). In multivariable analysis, non-advanced SM (vs. advanced) remained independently associated with improved OS (hazard ratio = 0.44; p < 0.001).

conclusionsSM-AHN is associated with reduced survival compared with SM without AHN across SM subtypes. Outcomes in SM-AHN are driven primarily by the aggressiveness of the mastocytosis component, supporting recognition and classification of SM in patients with concomitant myeloid neoplasms, given approved KIT-targeted tyrosine kinase inhibitors for advanced SM.

Indexed as

Mastocytosis, SystemicMyeloproliferative DisordersAdultAgedAged, 80 and overEuropeFemaleHumansMaleMiddle AgedPrognosisRegistriesYoung AdultAcute myeloid leukemiaAssociated myeloid neoplasmChronic myelomonocytic leukemiaKIT D816VOverall survivalSM-AHNSystemic mastocytosis

Identifiers

PMID42363232
PMCPMC13536581

What Socratic holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.