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

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....

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Published in:	Leukemia Vol. 36; no. 2; pp. 516 - 524
Main Authors:	Zanotti, Roberta, Bonifacio, Massimiliano, Lucchini, Giuseppe, Sperr, Wolfgang R., Scaffidi, Luigi, van Anrooij, Björn, Oude Elberink, Hanneke NC, Rossignol, Julien, Hermine, Olivier, Gorska, Aleksandra, Lange, Magdalena, Hadzijusufovic, Emir, Miething, Cornelius, Müller, Sabine, Perkins, Cecelia, Shomali, William, Elena, Chiara, Illerhaus, Anja, Jawhar, Mohamad, Parente, Roberta, Caroppo, Francesca, Solomianyi, Oleksii, Zink, Alexander, Mattsson, Mattias, Yavuz, Akif Selim, Panse, Jens, Varkonyi, Judit, Doubek, Michael, Sabato, Vito, Breynaert, Christine, Vucinic, Vladan, Schug, Tanja, Hägglund, Hans, Wortmann, Friederike, Brockow, Knut, Angelova-Fischer, Irena, Belloni Fortina, Anna, Triggiani, Massimo, Reiter, Andreas, Hartmann, Karin, Malcovati, Luca, Gotlib, Jason, Shoumariyeh, Khalid, Niedoszytko, Marek, Arock, Michel, Kluin-Nelemans, Hanneke C., Bonadonna, Patrizia, Valent, Peter
Format:	Journal Article
Language:	English
Published:	London Nature Publishing Group UK 01-02-2022 Nature Publishing Group
Subjects:	631/67/1990/2331 692/308/174 692/699/1541/1990/2331 Adult Aged Aged, 80 and over Allergic reactions Bone marrow Bone Marrow - metabolism Bone Marrow - pathology Cancer Research Criteria Critical Care Medicine Diagnostic systems Europe - epidemiology Female Follow-Up Studies Hematology Humans Hypersensitivity Intensive Internal Medicine Lesions Male Mast cells Mast Cells - metabolism Mast Cells - pathology Mastocytosis Mastocytosis - diagnosis Mastocytosis - epidemiology Mastocytosis - metabolism Mastocytosis, Systemic - diagnosis Mastocytosis, Systemic - epidemiology Mastocytosis, Systemic - metabolism Medical diagnosis Medicine Medicine & Public Health Middle Aged Oncology Prognosis Risk analysis Risk factors Skin diseases Skin Diseases - physiopathology Skin lesions Survival Survival Rate Tryptase Tryptases - metabolism Europe
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Summary:	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.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0887-6924 1476-5551 1476-5551
DOI:	10.1038/s41375-021-01406-y