Clinical features and survival of patients with indolent
systemic mastocytosis defined by the updated WHO classification

J 2020

Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification

TRIZULJAK, Jakub, W. R. SPERR, Lucie NEKVINDOVA, H. O. ELBERINK, K. V. GLEIXNER et. al.

Basic information

Original name

Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification

Authors

TRIZULJAK, Jakub (703 Slovakia, belonging to the institution), W. R. SPERR, Lucie NEKVINDOVA (203 Czech Republic), H. O. ELBERINK, K. V. GLEIXNER, A. GORSKA, M. LANGE, K. HARTMANN, A. ILLERHAUS, M. BONIFACIO, C. PERKINS, C. ELENA, L. MALCOVATI, A. B. FORTINA, K. SHOUMARIYEH, M. JAWHAR, R. ZANOTTI, P. BONADONNA, F. CAROPPO, A. ZINK, M. TRIGGIANI, R. PARENTE, Bubnoff N. VON, A. S. YAVUZ, H. HAGGLUND, M. MATTSSON, J. PANSE, N. JAEKEL, A. KILBERTUS, O. HERMINE, M. AROCK, D. FUCHS, V. SABATO, K. BROCKOW, A. BRETTERKLIEBER, M. NIEDOSZYTKO, Anrooij B. VAN, A. REITER, J. GOTLIB, H. C. KLUIN-NELEMANS, Jiří MAYER (203 Czech Republic, belonging to the institution), Michael DOUBEK (203 Czech Republic, guarantor, belonging to the institution) and P. VALENT

Edition

Allergy, Hoboken, Wiley, 2020, 0105-4538

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30102 Immunology

Country of publisher

United States of America

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

URL

Impact factor

Impact factor: 13.146

RIV identification code

RIV/00216224:14110/20:00114918

Organization unit

Faculty of Medicine

DOI

http://dx.doi.org/10.1111/all.14248

UT WoS

000552392800006

Keywords in English

cutaneous mastocytosis; indolent systemic mastocytosis; prognostication; survival; WHO classification

Abstract

V originále

Background: In indolent systemic mastocytosis (ISM), several risk factors of disease progression have been identified. Previous studies, performed with limited patient numbers, have also shown that the clinical course in ISM is stable and comparable to that of cutaneous mastocytosis (CM). The aim of this project was to compare the prognosis of patients with ISM with that of patients with CM. Methods: We employed a dataset of 1993 patients from the registry of the European Competence Network on Mastocytosis (ECNM) to compare outcomes of ISM and CM. Results: We found that overall survival (OS) is worse in ISM compared to CM. Moreover, in patients with typical ISM, bone marrow mastocytosis (BMM), and smoldering SM (SSM), 4.1% of disease progressions have been observed (4.9% of progressions in typical ISM group, 1.7% in BMM, and 9.4% in SSM). Progressions to advanced SM were observed in 2.9% of these patients. In contrast, six patients with CM (1.7%) converted to ISM and no definitive progression to advanced SM was found. No significant differences in OS and event-free survival (EFS) were found when comparing ISM, BMM, and SSM. Higher risk of both progression and death was significantly associated with male gender, worse performance status, and organomegaly. Conclusion: Our data confirm the clinical impact of the WHO classification that separates ISM from CM and from other SM variants.

Links

LQ1601, research and development project

Name: CEITEC 2020 (Acronym: CEITEC2020)

Investor: Ministry of Education, Youth and Sports of the CR

TE02000058, research and development project

Name: Centrum kompetence pro molekulární diagnostiku a personalizovanou medicínu (Acronym: MOLDIMED)

Investor: Technology Agency of the Czech Republic

Citovat

TRIZULJAK, Jakub, W. R. SPERR, Lucie NEKVINDOVA, H. O. ELBERINK, K. V. GLEIXNER, A. GORSKA, M. LANGE, K. HARTMANN, A. ILLERHAUS, M. BONIFACIO, C. PERKINS, C. ELENA, L. MALCOVATI, A. B. FORTINA, K. SHOUMARIYEH, M. JAWHAR, R. ZANOTTI, P. BONADONNA, F. CAROPPO, A. ZINK, M. TRIGGIANI, R. PARENTE, Bubnoff N. VON, A. S. YAVUZ, H. HAGGLUND, M. MATTSSON, J. PANSE, N. JAEKEL, A. KILBERTUS, O. HERMINE, M. AROCK, D. FUCHS, V. SABATO, K. BROCKOW, A. BRETTERKLIEBER, M. NIEDOSZYTKO, Anrooij B. VAN, A. REITER, J. GOTLIB, H. C. KLUIN-NELEMANS, Jiří MAYER, Michael DOUBEK and P. VALENT. Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification. Allergy. Hoboken: Wiley, 2020, vol. 75, No 8, p. 1927-1938. ISSN 0105-4538. Available from: https://dx.doi.org/10.1111/all.14248.

@article{1743659,
   author = {Trizuljak, Jakub and Sperr, W. R. and Nekvindova, Lucie and Elberink, H. O. and Gleixner, K. V. and Gorska, A. and Lange, M. and Hartmann, K. and Illerhaus, A. and Bonifacio, M. and Perkins, C. and Elena, C. and Malcovati, L. and Fortina, A. B. and Shoumariyeh, K. and Jawhar, M. and Zanotti, R. and Bonadonna, P. and Caroppo, F. and Zink, A. and Triggiani, M. and Parente, R. and von, Bubnoff N. and Yavuz, A. S. and Hagglund, H. and Mattsson, M. and Panse, J. and Jaekel, N. and Kilbertus, A. and Hermine, O. and Arock, M. and Fuchs, D. and Sabato, V. and Brockow, K. and Bretterklieber, A. and Niedoszytko, M. and van, Anrooij B. and Reiter, A. and Gotlib, J. and KluinandNelemans, H. C. and Mayer, Jiří and Doubek, Michael and Valent, P.},
   article_location = {Hoboken},
   article_number = {8},
   doi = {http://dx.doi.org/10.1111/all.14248},
   keywords = {cutaneous mastocytosis; indolent systemic mastocytosis; prognostication; survival; WHO classification},
   language = {eng},
   issn = {0105-4538},
   journal = {Allergy},
   title = {Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification},
   url = {https://onlinelibrary.wiley.com/doi/full/10.1111/all.14248},
   volume = {75},
   year = {2020}
}

TY  - JOUR
ID  - 1743659
AU  - Trizuljak, Jakub - Sperr, W. R. - Nekvindova, Lucie - Elberink, H. O. - Gleixner, K. V. - Gorska, A. - Lange, M. - Hartmann, K. - Illerhaus, A. - Bonifacio, M. - Perkins, C. - Elena, C. - Malcovati, L. - Fortina, A. B. - Shoumariyeh, K. - Jawhar, M. - Zanotti, R. - Bonadonna, P. - Caroppo, F. - Zink, A. - Triggiani, M. - Parente, R. - von, Bubnoff N. - Yavuz, A. S. - Hagglund, H. - Mattsson, M. - Panse, J. - Jaekel, N. - Kilbertus, A. - Hermine, O. - Arock, M. - Fuchs, D. - Sabato, V. - Brockow, K. - Bretterklieber, A. - Niedoszytko, M. - van, Anrooij B. - Reiter, A. - Gotlib, J. - Kluin-Nelemans, H. C. - Mayer, Jiří - Doubek, Michael - Valent, P.
PY  - 2020
TI  - Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification
JF  - Allergy
VL  - 75
IS  - 8
SP  - 1927-1938
EP  - 1927-1938
PB  - Wiley
SN  - 01054538
KW  - cutaneous mastocytosis
KW  - indolent systemic mastocytosis
KW  - prognostication
KW  - survival
KW  - WHO classification
UR  - https://onlinelibrary.wiley.com/doi/full/10.1111/all.14248
L2  - https://onlinelibrary.wiley.com/doi/full/10.1111/all.14248
N2  - Background: In indolent systemic mastocytosis (ISM), several risk factors of disease progression have been identified. Previous studies, performed with limited patient numbers, have also shown that the clinical course in ISM is stable and comparable to that of cutaneous mastocytosis (CM). The aim of this project was to compare the prognosis of patients with ISM with that of patients with CM. Methods: We employed a dataset of 1993 patients from the registry of the European Competence Network on Mastocytosis (ECNM) to compare outcomes of ISM and CM. Results: We found that overall survival (OS) is worse in ISM compared to CM. Moreover, in patients with typical ISM, bone marrow mastocytosis (BMM), and smoldering SM (SSM), 4.1% of disease progressions have been observed (4.9% of progressions in typical ISM group, 1.7% in BMM, and 9.4% in SSM). Progressions to advanced SM were observed in 2.9% of these patients. In contrast, six patients with CM (1.7%) converted to ISM and no definitive progression to advanced SM was found. No significant differences in OS and event-free survival (EFS) were found when comparing ISM, BMM, and SSM. Higher risk of both progression and death was significantly associated with male gender, worse performance status, and organomegaly. Conclusion: Our data confirm the clinical impact of the WHO classification that separates ISM from CM and from other SM variants.
ER  -

TRIZULJAK, Jakub, W. R. SPERR, Lucie NEKVINDOVA, H. O. ELBERINK, K. V. GLEIXNER, A. GORSKA, M. LANGE, K. HARTMANN, A. ILLERHAUS, M. BONIFACIO, C. PERKINS, C. ELENA, L. MALCOVATI, A. B. FORTINA, K. SHOUMARIYEH, M. JAWHAR, R. ZANOTTI, P. BONADONNA, F. CAROPPO, A. ZINK, M. TRIGGIANI, R. PARENTE, Bubnoff N. VON, A. S. YAVUZ, H. HAGGLUND, M. MATTSSON, J. PANSE, N. JAEKEL, A. KILBERTUS, O. HERMINE, M. AROCK, D. FUCHS, V. SABATO, K. BROCKOW, A. BRETTERKLIEBER, M. NIEDOSZYTKO, Anrooij B. VAN, A. REITER, J. GOTLIB, H. C. KLUIN-NELEMANS, Jiří MAYER, Michael DOUBEK and P. VALENT. Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification. \textit{Allergy}. Hoboken: Wiley, 2020, vol.~75, No~8, p.~1927-1938. ISSN~0105-4538. Available from: https://dx.doi.org/10.1111/all.14248.

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