Zanotti, Roberta, Bonifacio, Massimiliano, Lucchini, Giuseppe, Sperr, Wolfgang R., Scaffidi, Luigi, van Anrooij, Bjoern, Elberink, Hanneke N. C. Oude, Rossignol, Julien, Hermine, Olivier, Gorska, Aleksandra, Lange, Magdalena, Hadzijusufovic, Emir ORCID: 0000-0001-7409-4204, Miething, Cornelius, Muller, Sabine, Perkins, Cecelia, Shomali, William, Elena, Chiara, Illerhaus, Anja, Jawhar, Mohamad, Parente, Roberta, Caroppo, Francesca, Solomianyi, Oleksii, Zink, Alexander ORCID: 0000-0001-9313-6588, Mattsson, Mattias, Yavuz, Akif Selim, Panse, Jens, Varkonyi, Judit, Doubek, Michael, Sabato, Vito, Breynaert, Christine ORCID: 0000-0002-7368-1068, Vucinic, Vladan, Schug, Tanja, Hagglund, Hans, Wortmann, Friederike, Brockow, Knut, Angelova-Fischer, Irena, Fortina, Anna Belloni ORCID: 0000-0001-5791-0775, Triggiani, Massimo, Reiter, Andreas, Hartmann, Karin ORCID: 0000-0002-4595-8226, Malcovati, Luca, Gotlib, Jason, Shoumariyeh, Khalid, Niedoszytko, Marek, Arock, Michel, Kluin-Nelemans, Hanneke C., Bonadonna, Patrizia and Valent, Peter (2022). Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis. Leukemia, 36 (2). S. 516 - 525. LONDON: SPRINGERNATURE. ISSN 1476-5551

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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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Zanotti, RobertaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bonifacio, MassimilianoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lucchini, GiuseppeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sperr, Wolfgang R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Scaffidi, LuigiUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
van Anrooij, BjoernUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Elberink, Hanneke N. C. OudeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rossignol, JulienUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hermine, OlivierUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gorska, AleksandraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lange, MagdalenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hadzijusufovic, EmirUNSPECIFIEDorcid.org/0000-0001-7409-4204UNSPECIFIED
Miething, CorneliusUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Muller, SabineUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Perkins, CeceliaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Shomali, WilliamUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Elena, ChiaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Illerhaus, AnjaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jawhar, MohamadUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Parente, RobertaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Caroppo, FrancescaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Solomianyi, OleksiiUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zink, AlexanderUNSPECIFIEDorcid.org/0000-0001-9313-6588UNSPECIFIED
Mattsson, MattiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Yavuz, Akif SelimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Panse, JensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Varkonyi, JuditUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Doubek, MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sabato, VitoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Breynaert, ChristineUNSPECIFIEDorcid.org/0000-0002-7368-1068UNSPECIFIED
Vucinic, VladanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schug, TanjaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hagglund, HansUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wortmann, FriederikeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Brockow, KnutUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Angelova-Fischer, IrenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fortina, Anna BelloniUNSPECIFIEDorcid.org/0000-0001-5791-0775UNSPECIFIED
Triggiani, MassimoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Reiter, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hartmann, KarinUNSPECIFIEDorcid.org/0000-0002-4595-8226UNSPECIFIED
Malcovati, LucaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gotlib, JasonUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Shoumariyeh, KhalidUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Niedoszytko, MarekUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Arock, MichelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kluin-Nelemans, Hanneke C.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bonadonna, PatriziaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Valent, PeterUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-607770
DOI: 10.1038/s41375-021-01406-y
Journal or Publication Title: Leukemia
Volume: 36
Number: 2
Page Range: S. 516 - 525
Date: 2022
Publisher: SPRINGERNATURE
Place of Publication: LONDON
ISSN: 1476-5551
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
MAST-CELL DISORDERS; SYSTEMIC MASTOCYTOSIS; C-KIT; CLASSIFICATION; MUTATIONS; MARKERS; SURVIVAL; BURDENMultiple languages
Oncology; HematologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/60777

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