Serum chemistry profiling and prognostication in systemic
mastocytosis: a registry-based study of the ECNM and GREM

J 2024

Serum chemistry profiling and prognostication in systemic mastocytosis: a registry-based study of the ECNM and GREM

LUEBKE, Johannes, Alicia SCHMID, Deborah CHRISTEN, Hanneke N G Oude ELBERINK, Lambert F R SPAN et. al.

Basic information

Original name

Serum chemistry profiling and prognostication in systemic mastocytosis: a registry-based study of the ECNM and GREM

Authors

LUEBKE, Johannes, Alicia SCHMID, Deborah CHRISTEN, Hanneke N G Oude ELBERINK, Lambert F R SPAN, Marek NIEDOSZYTKO, Aleksandra GORSKA, Magdalena LANGE, Karoline V GLEIXNER, Emir HADZIJUSUFOVIC, Alex STEFAN, Irena ANGELOVA-FISCHER, Roberta ZANOTTI, Massimiliano BONIFACIO, Patrizia BONADONNA, Khalid SHOUMARIYEH, von Bubnoff NIKOLAS, Sabine MUELLER, Cecelia PERKINS, Chiara ELENA, Luca MALCOVATI, Hans HAGGLUND, Mattias MATTSSON, Roberta PARENTE, Judit VARKONYI, Anna Belloni FORTINA, Francesca CAROPPO, Knut BROCKOW, Alexander ZINK, Christine BREYNAERT, Toon LEVEN, Akif Selim YAVUZ, Michael DOUBEK (203 Czech Republic, belonging to the institution), Vito SABATO, Tanja SCHUG, Karin HARTMANN, Massimo TRIGGIANI, Jason GOTLIB, Olivier HERMINE, Michel AROCK, Hanneke C KLUIN-NELEMANS, Jens PANSE, Wolfgang R SPERR, Peter VALENT, Andreas REITER and Juliana SCHWAAB

Edition

Blood advances, AMSTERDAM, ELSEVIER, 2024, 2473-9529

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30205 Hematology

Country of publisher

Netherlands

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

URL

Impact factor

Impact factor: 7.500 in 2022

Organization unit

Faculty of Medicine

DOI

http://dx.doi.org/10.1182/bloodadvances.2024012756

UT WoS

001252795100001

Keywords in English

systemic mastocytosis; serum chemistry profiling

Abstract

V originále

Certain laboratory abnormalities correlate with subvariants of systemic mastocytosis (SM) and are often prognostically relevant. To assess the diagnostic and prognostic value of individual serum chemistry parameters in SM, 2607 patients enrolled within the European Competence Network on Mastocytosis and 575 patients enrolled within the German Registry on Eosinophils and Mast Cells were analyzed. For screening and diagnosis of SM, tryptase was identified as the most speci fic serum parameter. For differentiation between indolent and advanced SM (AdvSM), the following serum parameters were most relevant: tryptase, alkaline phosphatase, beta 2-microglobulin, lactate dehydrogenase (LDH), albumin, vitamin B12, and C-reactive protein (P < .001). With regard to subvariants of AdvSM, an elevated LDH of >= 260 U/L was associated with multilineage expansion (leukocytosis, r = 0.37, P < .001; monocytosis, r = 0.26, P < .001) and the presence of an associated myeloid neoplasm (P < .001), whereas tryptase levels were highest in mast cell leukemia (MCL) vs non-MCL (308 mu g/L vs 146 mu g/L, P = .003). Based on multivariable analysis, the hazard-risk weighted assignment of 1 point to LDH (hazard ratio [HR], 2.1; 95% confidence interval [CI], 1.1-4.0; P = .018) and 1.5 points each to beta 2-microglobulin (HR, 2.7; 95% CI, 1.4-5.4; P = .004) and albumin (HR, 3.3; 95% CI, 1.7-6.5; P = .001) delineated a highly predictive 3-tier risk classification system (0 points, 8.1 years vs 1 point, 2.5 years; >= 1.5 points, 1.7 years; P < .001). Moreover, serum chemistry parameters enabled further stratification of patients classified as having an International Prognostic Scoring System for Mastocytosis-AdvSM1/2 risk score (P = .027). In conclusion, serum chemistry pro filing is a crucial tool in the clinical practice supporting diagnosis and prognostication of SM and its subvariants.

Citovat

LUEBKE, Johannes, Alicia SCHMID, Deborah CHRISTEN, Hanneke N G Oude ELBERINK, Lambert F R SPAN, Marek NIEDOSZYTKO, Aleksandra GORSKA, Magdalena LANGE, Karoline V GLEIXNER, Emir HADZIJUSUFOVIC, Alex STEFAN, Irena ANGELOVA-FISCHER, Roberta ZANOTTI, Massimiliano BONIFACIO, Patrizia BONADONNA, Khalid SHOUMARIYEH, von Bubnoff NIKOLAS, Sabine MUELLER, Cecelia PERKINS, Chiara ELENA, Luca MALCOVATI, Hans HAGGLUND, Mattias MATTSSON, Roberta PARENTE, Judit VARKONYI, Anna Belloni FORTINA, Francesca CAROPPO, Knut BROCKOW, Alexander ZINK, Christine BREYNAERT, Toon LEVEN, Akif Selim YAVUZ, Michael DOUBEK, Vito SABATO, Tanja SCHUG, Karin HARTMANN, Massimo TRIGGIANI, Jason GOTLIB, Olivier HERMINE, Michel AROCK, Hanneke C KLUIN-NELEMANS, Jens PANSE, Wolfgang R SPERR, Peter VALENT, Andreas REITER and Juliana SCHWAAB. Serum chemistry profiling and prognostication in systemic mastocytosis: a registry-based study of the ECNM and GREM. Blood advances. AMSTERDAM: ELSEVIER, 2024, vol. 8, No 11, p. 2890-2900. ISSN 2473-9529. Available from: https://dx.doi.org/10.1182/bloodadvances.2024012756.

@article{2418100,
   author = {Luebke, Johannes and Schmid, Alicia and Christen, Deborah and Elberink, Hanneke N G Oude and Span, Lambert F R and Niedoszytko, Marek and Gorska, Aleksandra and Lange, Magdalena and Gleixner, Karoline V and Hadzijusufovic, Emir and Stefan, Alex and AngelovaandFischer, Irena and Zanotti, Roberta and Bonifacio, Massimiliano and Bonadonna, Patrizia and Shoumariyeh, Khalid and Nikolas, von Bubnoff and Mueller, Sabine and Perkins, Cecelia and Elena, Chiara and Malcovati, Luca and Hagglund, Hans and Mattsson, Mattias and Parente, Roberta and Varkonyi, Judit and Fortina, Anna Belloni and Caroppo, Francesca and Brockow, Knut and Zink, Alexander and Breynaert, Christine and Leven, Toon and Yavuz, Akif Selim and Doubek, Michael and Sabato, Vito and Schug, Tanja and Hartmann, Karin and Triggiani, Massimo and Gotlib, Jason and Hermine, Olivier and Arock, Michel and KluinandNelemans, Hanneke C and Panse, Jens and Sperr, Wolfgang R and Valent, Peter and Reiter, Andreas and Schwaab, Juliana},
   article_location = {AMSTERDAM},
   article_number = {11},
   doi = {http://dx.doi.org/10.1182/bloodadvances.2024012756},
   keywords = {systemic mastocytosis; serum chemistry profiling},
   language = {eng},
   issn = {2473-9529},
   journal = {Blood advances},
   title = {Serum chemistry profiling and prognostication in systemic mastocytosis: a registry-based study of the ECNM and GREM},
   url = {https://ashpublications.org/bloodadvances/article/8/11/2890/515667/Serum-chemistry-profiling-and-prognostication-in},
   volume = {8},
   year = {2024}
}

TY  - JOUR
ID  - 2418100
AU  - Luebke, Johannes - Schmid, Alicia - Christen, Deborah - Elberink, Hanneke N G Oude - Span, Lambert F R - Niedoszytko, Marek - Gorska, Aleksandra - Lange, Magdalena - Gleixner, Karoline V - Hadzijusufovic, Emir - Stefan, Alex - Angelova-Fischer, Irena - Zanotti, Roberta - Bonifacio, Massimiliano - Bonadonna, Patrizia - Shoumariyeh, Khalid - Nikolas, von Bubnoff - Mueller, Sabine - Perkins, Cecelia - Elena, Chiara - Malcovati, Luca - Hagglund, Hans - Mattsson, Mattias - Parente, Roberta - Varkonyi, Judit - Fortina, Anna Belloni - Caroppo, Francesca - Brockow, Knut - Zink, Alexander - Breynaert, Christine - Leven, Toon - Yavuz, Akif Selim - Doubek, Michael - Sabato, Vito - Schug, Tanja - Hartmann, Karin - Triggiani, Massimo - Gotlib, Jason - Hermine, Olivier - Arock, Michel - Kluin-Nelemans, Hanneke C - Panse, Jens - Sperr, Wolfgang R - Valent, Peter - Reiter, Andreas - Schwaab, Juliana
PY  - 2024
TI  - Serum chemistry profiling and prognostication in systemic mastocytosis: a registry-based study of the ECNM and GREM
JF  - Blood advances
VL  - 8
IS  - 11
SP  - 2890-2900
EP  - 2890-2900
PB  - ELSEVIER
SN  - 24739529
KW  - systemic mastocytosis
KW  - serum chemistry profiling
UR  - https://ashpublications.org/bloodadvances/article/8/11/2890/515667/Serum-chemistry-profiling-and-prognostication-in
N2  - Certain laboratory abnormalities correlate with subvariants of systemic mastocytosis (SM) and are often prognostically relevant. To assess the diagnostic and prognostic value of individual serum chemistry parameters in SM, 2607 patients enrolled within the European Competence Network on Mastocytosis and 575 patients enrolled within the German Registry on Eosinophils and Mast Cells were analyzed. For screening and diagnosis of SM, tryptase was identified as the most speci fic serum parameter. For differentiation between indolent and advanced SM (AdvSM), the following serum parameters were most relevant: tryptase, alkaline phosphatase, beta 2-microglobulin, lactate dehydrogenase (LDH), albumin, vitamin B12, and C-reactive protein (P < .001). With regard to subvariants of AdvSM, an elevated LDH of >= 260 U/L was associated with multilineage expansion (leukocytosis, r = 0.37, P < .001; monocytosis, r = 0.26, P < .001) and the presence of an associated myeloid neoplasm (P < .001), whereas tryptase levels were highest in mast cell leukemia (MCL) vs non-MCL (308 mu g/L vs 146 mu g/L, P = .003). Based on multivariable analysis, the hazard-risk weighted assignment of 1 point to LDH (hazard ratio [HR], 2.1; 95% confidence interval [CI], 1.1-4.0; P = .018) and 1.5 points each to beta 2-microglobulin (HR, 2.7; 95% CI, 1.4-5.4; P = .004) and albumin (HR, 3.3; 95% CI, 1.7-6.5; P = .001) delineated a highly predictive 3-tier risk classification system (0 points, 8.1 years vs 1 point, 2.5 years; >= 1.5 points, 1.7 years; P < .001). Moreover, serum chemistry parameters enabled further stratification of patients classified as having an International Prognostic Scoring System for Mastocytosis-AdvSM1/2 risk score (P = .027). In conclusion, serum chemistry pro filing is a crucial tool in the clinical practice supporting diagnosis and prognostication of SM and its subvariants.
ER  -

LUEBKE, Johannes, Alicia SCHMID, Deborah CHRISTEN, Hanneke N G Oude ELBERINK, Lambert F R SPAN, Marek NIEDOSZYTKO, Aleksandra GORSKA, Magdalena LANGE, Karoline V GLEIXNER, Emir HADZIJUSUFOVIC, Alex STEFAN, Irena ANGELOVA-FISCHER, Roberta ZANOTTI, Massimiliano BONIFACIO, Patrizia BONADONNA, Khalid SHOUMARIYEH, von Bubnoff NIKOLAS, Sabine MUELLER, Cecelia PERKINS, Chiara ELENA, Luca MALCOVATI, Hans HAGGLUND, Mattias MATTSSON, Roberta PARENTE, Judit VARKONYI, Anna Belloni FORTINA, Francesca CAROPPO, Knut BROCKOW, Alexander ZINK, Christine BREYNAERT, Toon LEVEN, Akif Selim YAVUZ, Michael DOUBEK, Vito SABATO, Tanja SCHUG, Karin HARTMANN, Massimo TRIGGIANI, Jason GOTLIB, Olivier HERMINE, Michel AROCK, Hanneke C KLUIN-NELEMANS, Jens PANSE, Wolfgang R SPERR, Peter VALENT, Andreas REITER and Juliana SCHWAAB. Serum chemistry profiling and prognostication in systemic mastocytosis: a registry-based study of the ECNM and GREM. \textit{Blood advances}. AMSTERDAM: ELSEVIER, 2024, vol.~8, No~11, p.~2890-2900. ISSN~2473-9529. Available from: https://dx.doi.org/10.1182/bloodadvances.2024012756.

Detailed Information on Publication Record