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CHRONIC MYELOPROLIFERATIVE NEOPLASMS

Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis

Leukemia volume 36, pages 516–524 (2022)Cite this article

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Abstract

In the current classification of the World Health Organization (WHO), bone marrow mastocytosis (BMM) is a provisional variant of indolent systemic mastocytosis (ISM) defined by bone marrow involvement and absence of skin lesions. However, no additional diagnostic criteria for BMM have been proposed. Within the registry dataset of the European Competence Network on Mastocytosis, we compared characteristics and outcomes of 390 patients with BMM and 1175 patients with typical ISM. BMM patients were significantly older, predominantly male, had lower tryptase and lower burden of neoplastic mast cells, and displayed a higher frequency of allergic reactions, mainly triggered by Hymenoptera, than patients with typical ISM. The estimated 10-year progression-free survival of BMM and typical ISM was 95.9% and 92.6%, respectively. In BMM patients defined by WHO-based criteria, the presence of one B-Finding and tryptase level ≥125 ng/mL were identified as risk factors for progression in multivariate analyses. BMM patients without any of these risk factors were found to have better progression-free survival (p < 0.05) and better overall survival (p < 0.05) than other ISM patients. These data support the proposal to define BMM as a separate SM variant characterized by SM criteria, absence of skin lesions, absence of B-Findings, and tryptase levels <125 ng/mL.

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Fig. 1: Bone marrow histology of two patients with a diagnosis of D816V-mutated bone marrow mastocytosis included in this series.
Fig. 2: Long-term outcome of 300 patients with WHO-based bone marrow mastocytosis (BMM) and 859 patients with typical indolent systemic mastocytosis (ISMtyp).
Fig. 3: Prognosis of WHO-based bone marrow mastocytosis (BMM) according to risk.
Fig. 4: Prognostic comparison of patients with low-risk bone marrow mastocytosis (BMM) and other subgroups.

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Acknowledgements

The Authors gratefully acknowledge the contribution of Dr. Alice Parisi (Department of Diagnostics and Public Health, Section of Pathological Anatomy, University of Verona) for providing the histological images.

Funding

PV was supported by the Austrian Science Fund (FWF) grants P32470-B and F4704-B20. LM and CE were supported by the Associazione Italiana per la Ricerca sul Cancro (AIRC), Milan, Italy (Investigator Grant #20125; AIRC 5×1000 project #21267, International Accelerator project #22796).

Author information

Authors and Affiliations

  1. Department of Medicine, Section of Hematology, University of Verona, Verona, Italy

    Roberta Zanotti, Massimiliano Bonifacio & Luigi Scaffidi

  2. Biostatistical Service ASST of Mantova, Mantua, Italy

    Giuseppe Lucchini

  3. Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria

    Wolfgang R. Sperr, Emir Hadzijusufovic & Peter Valent

  4. Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria

    Wolfgang R. Sperr, Emir Hadzijusufovic & Peter Valent

  5. Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

    Björn van Anrooij & Hanneke C. Kluin-Nelemans

  6. Department of Allergology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

    Björn van Anrooij & Hanneke NC Oude Elberink

  7. Institut Imagine INSERM Unité 1163 and Centre National de la Recherche Scientifique ERL8654, Centre de Reference des Mastocytoses, University of Paris, Paris, France

    Julien Rossignol & Olivier Hermine

  8. Department of Allergology, Medical University of Gdańsk, Gdańsk, Poland

    Aleksandra Gorska & Marek Niedoszytko

  9. Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, Gdańsk, Poland

    Magdalena Lange

  10. Department/Hospital for Companion Animals and Horses, University Hospital for Small Animals, Internal Medicine Small Animals, University of Veterinary Medicine Vienna, Vienna, Austria

    Emir Hadzijusufovic

  11. Department of Medicine I, Medical Center, University of Freiburg, Faculty of Medicine, University of Freiburg, Germany and German Cancer Consortium (DKTK), Partner Site Freiburg, Freiburg, Germany

    Cornelius Miething, Sabine Müller & Khalid Shoumariyeh

  12. Division of Hematology, Department of Medicine, Stanford University School of Medicine/Stanford Cancer Institute, Stanford, CA, USA

    Cecelia Perkins, William Shomali & Jason Gotlib

  13. Division of Hematology, Fondazione IRCCS San Matteo, Pavia, Italy

    Chiara Elena & Luca Malcovati

  14. Department of Dermatology, University of Cologne, Cologne, Germany

    Anja Illerhaus

  15. Hämatologie und Onkologie, III. Medizinische Klinik, Universitätsmedizin Mannheim, Universität Heidelberg, Mannheim, Germany

    Mohamad Jawhar & Andreas Reiter

  16. Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy

    Roberta Parente & Massimo Triggiani

  17. Pediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy

    Francesca Caroppo & Anna Belloni Fortina

  18. Department of Internal Medicine 3, Hematology and Oncology, Kepler University Hospital, Johannes Kepler University, Linz, Austria

    Oleksii Solomianyi

  19. Department of Dermatology and Allergy Biederstein, Technical University of Munich, Munich, Germany

    Alexander Zink & Knut Brockow

  20. Department of Medical Sciences, Uppsala University and Section of Hematology, Uppsala University Hospital, Uppsala, Sweden

    Mattias Mattsson & Hans Hägglund

  21. Division of Hematology, Istanbul Medical School, University of Istanbul, Istanbul, Turkey

    Akif Selim Yavuz

  22. Department of Oncology, Haematology, Haemostaseology and Stem Cell Transplantation, University Hospital RWTH Aachen, Aachen, Germany

    Jens Panse

  23. Department of Hematology and Internal Medicine, Semmelweis University, Budapest, Hungary

    Judit Varkonyi

  24. University Hospital, Department of Hematology and Oncology, and Department of Medical Genetics and Genomics, Faculty of Medicine Masaryk University, Brno, Czech Republic

    Michael Doubek

  25. Faculty of Medicine and Health Sciences, Department of Immunology-Allergology-Rheumatology, University of Antwerp and Antwerp University Hospital, Antwerp, Belgium

    Vito Sabato

  26. KU Leuven Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group and MASTeL, University Hospitals Leuven, Leuven, Belgium

    Christine Breynaert

  27. Medical Clinic and Policlinic 1, Hematology and Cellular Therapy, Leipzig University Hospital, Leipzig, Germany

    Vladan Vucinic

  28. Department of Dermatology and Venereology, Medical University of Graz, Graz, Austria

    Tanja Schug

  29. Department of Hematology and Oncology, Medical Center, University of Schleswig-Holstein, Lübeck, Germany

    Friederike Wortmann

  30. Department of Dermatology and Venerology (AK), Kepler University Hospital, Johannes Kepler University, Linz, Austria

    Irena Angelova-Fischer

  31. Division of Allergy, Department of Dermatology, and Department of Biomedicine, University Hospital Basel and University of Basel, Basel, Switzerland

    Karin Hartmann

  32. Department of Molecular Medicine, University of Pavia, Pavia, Italy

    Luca Malcovati

  33. Laboratory of Hematology, Pitié-Salpêtrière Hospital, Paris, France

    Michel Arock

  34. Allergy Unit, Verona University Hospital, Verona, Italy

    Patrizia Bonadonna

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  1. Roberta Zanotti
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Contributions

RZ, MB, PB, PV designed the study, analyzed and interpreted data, and wrote the paper. LS, BvA, HOE, JR, OH, AG, ML, EH, CM, SM, CP, WS, CE, AI, MJ, RP, FC, OS, AZ, MM, ASY, JP, JV, MD, VS, CB, VV, TS, HH, FW, KB, IAF, ABF, MT, AR, KH, LM, JG, KS, MN, MA, HKN recruited participants and contributed to data collection. GL and WRS performed statistical analyses. All Authors contributed to the review of the paper. All Authors agreed to be accountable for all aspects of the work.

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Correspondence to Massimiliano Bonifacio.

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Zanotti, R., Bonifacio, M., Lucchini, G. et al. Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis. Leukemia 36, 516–524 (2022). https://doi.org/10.1038/s41375-021-01406-y

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