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

Author:

Lübke Johannes1ORCID,Schmid Alicia1,Christen Deborah23ORCID,Oude Elberink Hanneke N. G.4ORCID,Span Lambert F. R.5,Niedoszytko Marek6,Gorska Aleksandra6,Lange Magdalena7ORCID,Gleixner Karoline V.89,Hadzijusufovic Emir8910ORCID,Stefan Alex11,Angelova-Fischer Irena12,Zanotti Roberta13,Bonifacio Massimiliano13,Bonadonna Patrizia14,Shoumariyeh Khalid1516,von Bubnoff Nikolas151617ORCID,Müller Sabine18,Perkins Cecelia19,Elena Chiara20,Malcovati Luca20ORCID,Hagglund Hans21,Mattsson Mattias21,Parente Roberta22,Varkonyi Judit23,Fortina Anna Belloni24,Caroppo Francesca24ORCID,Brockow Knut25ORCID,Zink Alexander25ORCID,Breynaert Christine26ORCID,Leven Toon26,Yavuz Akif Selim27,Doubek Michael28,Sabato Vito29,Schug Tanja30,Hartmann Karin3132ORCID,Triggiani Massimo22ORCID,Gotlib Jason19,Hermine Olivier33,Arock Michel34,Kluin-Nelemans Hanneke C.5ORCID,Panse Jens23ORCID,Sperr Wolfgang R.89,Valent Peter89ORCID,Reiter Andreas1,Schwaab Juliana1

Affiliation:

1. 1Department of Hematology and Oncology, University Hospital Mannheim, Heidelberg University, Mannheim, Germany

2. 2Department of Oncology, Hematology, Hemostaseology and Stem Cell Transplantation, University Hospital RWTH Aachen, Aachen, Germany

3. 3Center for Integrated Oncology, Aachen Bonn Cologne Düsseldorf, Aachen, Germany

4. 4Department of Allergology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

5. 5Department of Hematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

6. 6Department of Allergology, Medical University of Gdańsk, Gdańsk, Poland

7. 7Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, Gdańsk, Poland

8. 8Division of Hematology & Hemostaseology, Department of Internal Medicine I, Medical University of Vienna, Vienna, Austria

9. 9Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria

10. 10Department/University Clinic for Companion Animals and Horses, Internal Medicine Small Animals, University Clinic for Small Animals, University of Veterinary Medicine, Vienna, Austria

11. 11University Clinic for Hematology and Oncology, Kepler University Hospital, Johannes Kepler University, Linz, Austria

12. 12Department of Dermatology and Venereology, Allergy Center, Kepler University Hospital, Johannes Kepler University, Linz, Austria

13. 13Department of Medicine, Section of Hematology, Verona University Hospital, Verona, Italy

14. 14Allergy Unit, Verona University Hospital, Verona, Italy

15. 15Department of Medicine I, Medical Center University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany

16. 16German Cancer Consortium (DKTK), partner site Freiburg and German Cancer Research Center (DKFZ), Heidelberg, Germany

17. 17Department of Hematology and Oncology, Medical Center, University of Schleswig-Holstein, Campus Lübeck, Lübeck, Germany

18. 18Department of Dermatology, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany

19. 19Stanford Cancer Institute, Stanford University School of Medicine/Stanford Cancer Institute, Stanford, CA

20. 20Hematology Unit, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy

21. 21Division of Hematology, Department of Medical Sciences Uppsala University, Uppsala, Sweden

22. 22Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy

23. 23Department of Hematology, Semmelweis University, Budapest, Hungary

24. 24Department of Medicine, Pediatric Dermatology Unit, University of Padova, Padova, Italy

25. 25Department of Dermatology and Allergy, School of Medicine, Technical University of Munich, Munich, Germany

26. 26KU Leuven Department of Microbiology, Immunology and Transplantation, Allergy and Clinical Immunology Research Group and MASTeL, University Hospitals Leuven, Leuven, Belgium

27. 27Division of Hematology, Istanbul Medical School, University of Istanbul, Istanbul, Turkey

28. 28Department of Internal Medicine - Hematology and Oncology, University Hospital Brno & Faculty of Medicine, Masaryk University, Brno, Czech Republic

29. 29Department of Immunology-Allergology-Rheumatology, Faculty of Medicine and Health Sciences, University of Antwerp and Antwerp University Hospital, Antwerpen, Belgium

30. 30Department of Dermatology and Venereology, University Hospital Graz, Graz, Austria

31. 31Division of Allergy, Department of Dermatology, University Hospital Basel and University of Basel, Basel, Switzerland

32. 32Department of Biomedicine, University Hospital Basel and University of Basel, Basel, Switzerland

33. 33French Reference Center for Mastocytosis, Hospital Necker, Assistance Publique Hôpitaux de Paris, Imagine Institute, University Paris Descartes, Paris, France

34. 34French Reference Center for Mastocytosis, Pitié-Salpêtrière Hospital, Assistance Publique Hôpitaux de Paris, University Paris Sorbonne, Paris, France

Abstract

Abstract 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 specific serum parameter. For differentiation between indolent and advanced SM (AdvSM), the following serum parameters were most relevant: tryptase, alkaline phosphatase, β2-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 (308μg/L vs 146μg/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 β2-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 profiling is a crucial tool in the clinical practice supporting diagnosis and prognostication of SM and its subvariants.

Publisher

American Society of Hematology

Cited by 2 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. Management of Advanced Systemic Mastocytosis: Clinical Challenges;Journal of Blood Medicine;2024-09

2. Systemic Mastocytosis: State of the Art;Current Hematologic Malignancy Reports;2024-08-27

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3