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

Autor: Zanotti R; Department of Medicine, Section of Hematology, University of Verona, Verona, Italy., Bonifacio M; Department of Medicine, Section of Hematology, University of Verona, Verona, Italy. massimiliano.bonifacio@univr.it., Lucchini G; Biostatistical Service ASST of Mantova, Mantua, Italy., Sperr WR; Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria.; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria., Scaffidi L; Department of Medicine, Section of Hematology, University of Verona, Verona, Italy., van Anrooij B; Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.; Department of Allergology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Oude Elberink HN; Department of Allergology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Rossignol J; Institut Imagine INSERM Unité 1163 and Centre National de la Recherche Scientifique ERL8654, Centre de Reference des Mastocytoses, University of Paris, Paris, France., Hermine O; Institut Imagine INSERM Unité 1163 and Centre National de la Recherche Scientifique ERL8654, Centre de Reference des Mastocytoses, University of Paris, Paris, France., Gorska A; Department of Allergology, Medical University of Gdańsk, Gdańsk, Poland., Lange M; Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, Gdańsk, Poland., Hadzijusufovic E; Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria.; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.; Department/Hospital for Companion Animals and Horses, University Hospital for Small Animals, Internal Medicine Small Animals, University of Veterinary Medicine Vienna, Vienna, Austria., Miething C; 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., Müller S; 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., Perkins C; Division of Hematology, Department of Medicine, Stanford University School of Medicine/Stanford Cancer Institute, Stanford, CA, USA., Shomali W; Division of Hematology, Department of Medicine, Stanford University School of Medicine/Stanford Cancer Institute, Stanford, CA, USA., Elena C; Division of Hematology, Fondazione IRCCS San Matteo, Pavia, Italy., Illerhaus A; Department of Dermatology, University of Cologne, Cologne, Germany., Jawhar M; Hämatologie und Onkologie, III. Medizinische Klinik, Universitätsmedizin Mannheim, Universität Heidelberg, Mannheim, Germany., Parente R; Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy., Caroppo F; Pediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy., Solomianyi O; Department of Internal Medicine 3, Hematology and Oncology, Kepler University Hospital, Johannes Kepler University, Linz, Austria., Zink A; Department of Dermatology and Allergy Biederstein, Technical University of Munich, Munich, Germany., Mattsson M; Department of Medical Sciences, Uppsala University and Section of Hematology, Uppsala University Hospital, Uppsala, Sweden., Yavuz AS; Division of Hematology, Istanbul Medical School, University of Istanbul, Istanbul, Turkey., Panse J; Department of Oncology, Haematology, Haemostaseology and Stem Cell Transplantation, University Hospital RWTH Aachen, Aachen, Germany., Varkonyi J; Department of Hematology and Internal Medicine, Semmelweis University, Budapest, Hungary., Doubek M; University Hospital, Department of Hematology and Oncology, and Department of Medical Genetics and Genomics, Faculty of Medicine Masaryk University, Brno, Czech Republic., Sabato V; Faculty of Medicine and Health Sciences, Department of Immunology-Allergology-Rheumatology, University of Antwerp and Antwerp University Hospital, Antwerp, Belgium., Breynaert C; KU Leuven Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group and MASTeL, University Hospitals Leuven, Leuven, Belgium., Vucinic V; Medical Clinic and Policlinic 1, Hematology and Cellular Therapy, Leipzig University Hospital, Leipzig, Germany., Schug T; Department of Dermatology and Venereology, Medical University of Graz, Graz, Austria., Hägglund H; Department of Medical Sciences, Uppsala University and Section of Hematology, Uppsala University Hospital, Uppsala, Sweden., Wortmann F; Department of Hematology and Oncology, Medical Center, University of Schleswig-Holstein, Lübeck, Germany., Brockow K; Department of Dermatology and Allergy Biederstein, Technical University of Munich, Munich, Germany., Angelova-Fischer I; Department of Dermatology and Venerology (AK), Kepler University Hospital, Johannes Kepler University, Linz, Austria., Belloni Fortina A; Pediatric Dermatology Unit, Department of Medicine, University of Padova, Padova, Italy., Triggiani M; Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy., Reiter A; Hämatologie und Onkologie, III. Medizinische Klinik, Universitätsmedizin Mannheim, Universität Heidelberg, Mannheim, Germany., Hartmann K; Division of Allergy, Department of Dermatology, and Department of Biomedicine, University Hospital Basel and University of Basel, Basel, Switzerland., Malcovati L; Division of Hematology, Fondazione IRCCS San Matteo, Pavia, Italy.; Department of Molecular Medicine, University of Pavia, Pavia, Italy., Gotlib J; Division of Hematology, Department of Medicine, Stanford University School of Medicine/Stanford Cancer Institute, Stanford, CA, USA., Shoumariyeh K; 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., Niedoszytko M; Department of Allergology, Medical University of Gdańsk, Gdańsk, Poland., Arock M; Laboratory of Hematology, Pitié-Salpêtrière Hospital, Paris, France., Kluin-Nelemans HC; Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Bonadonna P; Allergy Unit, Verona University Hospital, Verona, Italy., Valent P; Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria.; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.
Jazyk: angličtina
Zdroj: Leukemia [Leukemia] 2022 Feb; Vol. 36 (2), pp. 516-524. Date of Electronic Publication: 2021 Sep 20.
DOI: 10.1038/s41375-021-01406-y
Abstrakt: 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.
(© 2021. The Author(s), under exclusive licence to Springer Nature Limited.)
Databáze: MEDLINE
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