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

J 2022

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

ZANOTTI, R., M. BONIFACIO, G. LUCCHINI, W. R. SPERR, L. SCAFFIDI et. al.

Basic information

Original name

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

Authors

ZANOTTI, R., M. BONIFACIO (guarantor), G. LUCCHINI, W. R. SPERR, L. SCAFFIDI, B. VAN ANROOIJ, H. N. C. O. ELBERINK, J. ROSSIGNOL, O. HERMINE, A. GORSKA, M. LANGE, E. HADZIJUSUFOVIC, C. MIETHING, S. MULLER, C. PERKINS, W. SHOMALI, C. ELENA, A. ILLERHAUS, M. JAWHAR, R. PARENTE, F. CAROPPO, O. SOLOMIANYI, A. ZINK, M. MATTSSON, A. S. YAVUZ, J. PANSE, J. VARKONYI, Michael DOUBEK (203 Czech Republic, belonging to the institution), V. SABATO, C. BREYNAERT, V. VUCINIC, T. SCHUG, H. HAGGLUND, F. WORTMANN, K. BROCKOW, I. ANGELOVA-FISCHER, A. B. FORTINA, M. TRIGGIANI, A. REITER, K. HARTMANN, L. MALCOVATI, J. GOTLIB, K. SHOUMARIYEH, M. NIEDOSZYTKO, M. AROCK, H. C. KLUIN-NELEMANS, P. BONADONNA and P. VALENT

Edition

Leukemia, London, Nature Publishing Group, 2022, 0887-6924

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30205 Hematology

Country of publisher

United Kingdom of Great Britain and Northern Ireland

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

URL

Impact factor

Impact factor: 11.400

RIV identification code

RIV/00216224:14110/22:00125368

Organization unit

Faculty of Medicine

DOI

http://dx.doi.org/10.1038/s41375-021-01406-y

UT WoS

000698099800004

Keywords in English

bone marrow mastocytosis

Abstract

V originále

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.

Citovat

ZANOTTI, R., M. BONIFACIO, G. LUCCHINI, W. R. SPERR, L. SCAFFIDI, B. VAN ANROOIJ, H. N. C. O. ELBERINK, J. ROSSIGNOL, O. HERMINE, A. GORSKA, M. LANGE, E. HADZIJUSUFOVIC, C. MIETHING, S. MULLER, C. PERKINS, W. SHOMALI, C. ELENA, A. ILLERHAUS, M. JAWHAR, R. PARENTE, F. CAROPPO, O. SOLOMIANYI, A. ZINK, M. MATTSSON, A. S. YAVUZ, J. PANSE, J. VARKONYI, Michael DOUBEK, V. SABATO, C. BREYNAERT, V. VUCINIC, T. SCHUG, H. HAGGLUND, F. WORTMANN, K. BROCKOW, I. ANGELOVA-FISCHER, A. B. FORTINA, M. TRIGGIANI, A. REITER, K. HARTMANN, L. MALCOVATI, J. GOTLIB, K. SHOUMARIYEH, M. NIEDOSZYTKO, M. AROCK, H. C. KLUIN-NELEMANS, P. BONADONNA and P. VALENT. Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis. Leukemia. London: Nature Publishing Group, 2022, vol. 36, No 2, p. 516-524. ISSN 0887-6924. Available from: https://dx.doi.org/10.1038/s41375-021-01406-y.

@article{1834277,
   author = {Zanotti, R. and Bonifacio, M. and Lucchini, G. and Sperr, W. R. and Scaffidi, L. and van Anrooij, B. and Elberink, H. N. C. O. and Rossignol, J. and Hermine, O. and Gorska, A. and Lange, M. and Hadzijusufovic, E. and Miething, C. and Muller, S. and Perkins, C. and Shomali, W. and Elena, C. and Illerhaus, A. and Jawhar, M. and Parente, R. and Caroppo, F. and Solomianyi, O. and Zink, A. and Mattsson, M. and Yavuz, A. S. and Panse, J. and Varkonyi, J. and Doubek, Michael and Sabato, V. and Breynaert, C. and Vucinic, V. and Schug, T. and Hagglund, H. and Wortmann, F. and Brockow, K. and AngelovaandFischer, I. and Fortina, A. B. and Triggiani, M. and Reiter, A. and Hartmann, K. and Malcovati, L. and Gotlib, J. and Shoumariyeh, K. and Niedoszytko, M. and Arock, M. and KluinandNelemans, H. C. and Bonadonna, P. and Valent, P.},
   article_location = {London},
   article_number = {2},
   doi = {http://dx.doi.org/10.1038/s41375-021-01406-y},
   keywords = {bone marrow mastocytosis},
   language = {eng},
   issn = {0887-6924},
   journal = {Leukemia},
   title = {Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis},
   url = {https://www.nature.com/articles/s41375-021-01406-y},
   volume = {36},
   year = {2022}
}

TY  - JOUR
ID  - 1834277
AU  - Zanotti, R. - Bonifacio, M. - Lucchini, G. - Sperr, W. R. - Scaffidi, L. - van Anrooij, B. - Elberink, H. N. C. O. - Rossignol, J. - Hermine, O. - Gorska, A. - Lange, M. - Hadzijusufovic, E. - Miething, C. - Muller, S. - Perkins, C. - Shomali, W. - Elena, C. - Illerhaus, A. - Jawhar, M. - Parente, R. - Caroppo, F. - Solomianyi, O. - Zink, A. - Mattsson, M. - Yavuz, A. S. - Panse, J. - Varkonyi, J. - Doubek, Michael - Sabato, V. - Breynaert, C. - Vucinic, V. - Schug, T. - Hagglund, H. - Wortmann, F. - Brockow, K. - Angelova-Fischer, I. - Fortina, A. B. - Triggiani, M. - Reiter, A. - Hartmann, K. - Malcovati, L. - Gotlib, J. - Shoumariyeh, K. - Niedoszytko, M. - Arock, M. - Kluin-Nelemans, H. C. - Bonadonna, P. - Valent, P.
PY  - 2022
TI  - Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis
JF  - Leukemia
VL  - 36
IS  - 2
SP  - 516-524
EP  - 516-524
PB  - Nature Publishing Group
SN  - 08876924
KW  - bone marrow mastocytosis
UR  - https://www.nature.com/articles/s41375-021-01406-y
N2  - 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.
ER  -

ZANOTTI, R., M. BONIFACIO, G. LUCCHINI, W. R. SPERR, L. SCAFFIDI, B. VAN ANROOIJ, H. N. C. O. ELBERINK, J. ROSSIGNOL, O. HERMINE, A. GORSKA, M. LANGE, E. HADZIJUSUFOVIC, C. MIETHING, S. MULLER, C. PERKINS, W. SHOMALI, C. ELENA, A. ILLERHAUS, M. JAWHAR, R. PARENTE, F. CAROPPO, O. SOLOMIANYI, A. ZINK, M. MATTSSON, A. S. YAVUZ, J. PANSE, J. VARKONYI, Michael DOUBEK, V. SABATO, C. BREYNAERT, V. VUCINIC, T. SCHUG, H. HAGGLUND, F. WORTMANN, K. BROCKOW, I. ANGELOVA-FISCHER, A. B. FORTINA, M. TRIGGIANI, A. REITER, K. HARTMANN, L. MALCOVATI, J. GOTLIB, K. SHOUMARIYEH, M. NIEDOSZYTKO, M. AROCK, H. C. KLUIN-NELEMANS, P. BONADONNA and P. VALENT. Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis. \textit{Leukemia}. London: Nature Publishing Group, 2022, vol.~36, No~2, p.~516-524. ISSN~0887-6924. Available from: https://dx.doi.org/10.1038/s41375-021-01406-y.

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