The Serum Tryptase Test: an Emerging Robust Biomarker in Clinical Hematology

Overview

Journal Expert Rev Hematol

Specialty Hematology

Date 2014 Aug 30

PMID 25169217

Citations 22

Authors

Peter Valent

Wolfgang R Sperr

Karl Sotlar

Andreas Reiter

Cem Akin

Jason Gotlib

Hans-Peter Horny

Michel Arock

Affiliations

Soon will be listed here.

Abstract

During the past few years, a number of molecular markers have been developed in clinical hematology, most of them related to specific gene defects. However, there is also an unmet need to develop novel serologic parameters to improve diagnostics and prognostication in daily practice. Among these, the serum tryptase appears to be a most reliable biomarker of myeloid neoplasms. Elevated tryptase levels are found in subgroups of patients with mastocytosis, myelodysplastic syndrome, myeloproliferative neoplasm, acute myeloid leukemia, chronic myeloid leukemia and chronic eosinophilic leukemia. In these patients, the tryptase level is of diagnostic and/or prognostic significance. In mastocytosis, an elevated tryptase level is a minor criterion of systemic disease and in BCR-ABL1(+) chronic myeloid leukemia, elevated tryptase at diagnosis correlates with treatment responses and overall survival. In patients with elevated tryptase, the enzyme also serves as follow-up parameter and can be employed to measure treatment-responses. In the current article, we review and update the perspectives of tryptase and provide recommendations for use of this conventional biomarker in daily practice.

Citing Articles

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References

Tefferi A, Lasho T, Schwager S, Steensma D, Mesa R, Li C . The JAK2(V617F) tyrosine kinase mutation in myelofibrosis with myeloid metaplasia: lineage specificity and clinical correlates. Br J Haematol. 2005; 131(3):320-8. DOI: 10.1111/j.1365-2141.2005.05776.x. View

Sperr W, El-Samahi A, Kundi M, Girschikofsky M, Winkler S, Lutz D . Elevated tryptase levels selectively cluster in myeloid neoplasms: a novel diagnostic approach and screen marker in clinical haematology. Eur J Clin Invest. 2009; 39(10):914-23. DOI: 10.1111/j.1365-2362.2009.02184.x. View

Schwartz L, Metcalfe D, Miller J, Earl H, Sullivan T . Tryptase levels as an indicator of mast-cell activation in systemic anaphylaxis and mastocytosis. N Engl J Med. 1987; 316(26):1622-6. DOI: 10.1056/NEJM198706253162603. View

Schwaab J, Schnittger S, Sotlar K, Walz C, Fabarius A, Pfirrmann M . Comprehensive mutational profiling in advanced systemic mastocytosis. Blood. 2013; 122(14):2460-6. DOI: 10.1182/blood-2013-04-496448. View

Roche-Lestienne C, Lepers S, Soenen-Cornu V, Kahn J, Lai J, Hachulla E . Molecular characterization of the idiopathic hypereosinophilic syndrome (HES) in 35 French patients with normal conventional cytogenetics. Leukemia. 2005; 19(5):792-8. DOI: 10.1038/sj.leu.2403722. View

Johnson R, Savage N, Chiang T, Gotlib J, Cherry A, Arber D . Hidden mastocytosis in acute myeloid leukemia with t(8;21)(q22;q22). Am J Clin Pathol. 2013; 140(4):525-35. DOI: 10.1309/AJCP1Q0YSXEAHNKK. View

Sperr W, Jordan J, Baghestanian M, Kiener H, Samorapoompichit P, Semper H . Expression of mast cell tryptase by myeloblasts in a group of patients with acute myeloid leukemia. Blood. 2001; 98(7):2200-9. DOI: 10.1182/blood.v98.7.2200. View

Cools J, DeAngelo D, Gotlib J, Stover E, Legare R, Cortes J . A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med. 2003; 348(13):1201-14. DOI: 10.1056/NEJMoa025217. View

Bain B . Myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1. Haematologica. 2010; 95(5):696-8. PMC: 2864371. DOI: 10.3324/haematol.2009.021675. View

10.

Sperr W, Mitterbauer M, Mitterbauer G, Kundi M, Jager U, Lechner K . Quantitation of minimal residual disease in acute myeloid leukemia by tryptase monitoring identifies a group of patients with a high risk of relapse. Clin Cancer Res. 2005; 11(18):6536-43. DOI: 10.1158/1078-0432.CCR-05-0732. View

11.

Schwartz L, Irani A, ROLLER K, Castells M, Schechter N . Quantitation of histamine, tryptase, and chymase in dispersed human T and TC mast cells. J Immunol. 1987; 138(8):2611-5. View

12.

Cazzola M, Della Porta M, Malcovati L . The genetic basis of myelodysplasia and its clinical relevance. Blood. 2013; 122(25):4021-34. PMC: 3862275. DOI: 10.1182/blood-2013-09-381665. View

13.

Valent P, Horny H, Escribano L, Longley B, Li C, Schwartz L . Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leuk Res. 2001; 25(7):603-25. DOI: 10.1016/s0145-2126(01)00038-8. View

14.

Florian S, Esterbauer H, Binder T, Mullauer L, Haas O, Sperr W . Systemic mastocytosis (SM) associated with chronic eosinophilic leukemia (SM-CEL): detection of FIP1L1/PDGFRalpha, classification by WHO criteria, and response to therapy with imatinib. Leuk Res. 2006; 30(9):1201-5. DOI: 10.1016/j.leukres.2005.11.014. View

15.

Schwartz L, Sakai K, Bradford T, Ren S, Zweiman B, Worobec A . The alpha form of human tryptase is the predominant type present in blood at baseline in normal subjects and is elevated in those with systemic mastocytosis. J Clin Invest. 1995; 96(6):2702-10. PMC: 185977. DOI: 10.1172/JCI118337. View

16.

Cross N, Reiter A . Fibroblast growth factor receptor and platelet-derived growth factor receptor abnormalities in eosinophilic myeloproliferative disorders. Acta Haematol. 2008; 119(4):199-206. DOI: 10.1159/000140631. View

17.

Jordan J, Fritsche-Polanz R, Sperr W, Mitterbauer G, Fodinger M, Schernthaner G . A case of 'smouldering' mastocytosis with high mast cell burden, monoclonal myeloid cells, and C-KIT mutation Asp-816-Val. Leuk Res. 2001; 25(7):627-34. DOI: 10.1016/s0145-2126(01)00019-4. View

18.

Yeung D, Hughes T . Therapeutic targeting of BCR-ABL: prognostic markers of response and resistance mechanism in chronic myeloid leukaemia. Crit Rev Oncog. 2012; 17(1):17-30. DOI: 10.1615/critrevoncog.v17.i1.30. View

19.

Alvarez-Twose I, Zanotti R, Gonzalez-de-Olano D, Bonadonna P, Vega A, Matito A . Nonaggressive systemic mastocytosis (SM) without skin lesions associated with insect-induced anaphylaxis shows unique features versus other indolent SM. J Allergy Clin Immunol. 2013; 133(2):520-8. DOI: 10.1016/j.jaci.2013.06.020. View

20.

Welch J, Ley T, Link D, Miller C, Larson D, Koboldt D . The origin and evolution of mutations in acute myeloid leukemia. Cell. 2012; 150(2):264-78. PMC: 3407563. DOI: 10.1016/j.cell.2012.06.023. View