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. 2024 Sep;79(9):2470-2481.
doi: 10.1111/all.16132. Epub 2024 Apr 23.

Prevalence of hypersensitivity reactions in various forms of mastocytosis: A pilot study of 2485 adult patients with mastocytosis collected in the ECNM registry

Marek Niedoszytko  1 , Aleksandra Gorska  1 , Knut Brockow  2 , Patrizia Bonadonna  3 , Magdalena Lange  4 , Hanneke Kluin-Nelemans  5 , Hanneke Oude-Elberink  6 , Vito Sabato  7 , Khalid Shoumariyeh  8 , Dagmar von Bubnoff  9 , Sabine Müller  10 , Anja Illerhaus  11 , Michael Doubek  12 , Irena Angelova-Fischer  13 , Olivier Hermine  14 , Michel Arock  15 , Chiara Elena  16 , Luca Malcovati  16   17 , Akif Selim Yavuz  18 , Tanja Daniela Schug  19 , Anna Belloni Fortina  20 , Várkonyi Judit  21 , Jason Gotlib  22 , Jens Panse  23 , Vladan Vucinic  24 , Andreas Reiter  25 , Juliana Schwaab  25 , Massimo Triggiani  26 , Mattias Mattsson  27 , Christine Breynaert  28 , Jan Romantowski  1 , Roberta Zanotti  29 , Elisa Olivieri  3 , Alexander Zink  2 , Annick van de Ven  6 , Alex Stefan  30 , Stephane Barete  14 , Francesca Caroppo  20 , Cecelia Perkins  22 , Vanessa Kennedy  22 , Deborah Christen  23 , Mohamad Jawhar  25   31 , Johannes Luebke  25 , Roberta Parente  26 , Kerstin Levedahl  27   32 , Emir Hadzijusufovic  33   34 , Karin Hartmann  35 , Boguslaw Nedoszytko  4   36 , Wolfgang R Sperr  33   34 , Peter Valent  33   34
Affiliations

Prevalence of hypersensitivity reactions in various forms of mastocytosis: A pilot study of 2485 adult patients with mastocytosis collected in the ECNM registry

Marek Niedoszytko et al. Allergy. 2024 Sep.

Abstract

Background: Hypersensitivity reactions (HR) are common in mastocytosis. However, little is known about triggers and risk factors. The registry of the European Competence Network on Mastocytosis (ECNM) enables reliable studies in a larger cohort of mastocytosis patients. We assessed prevalence, triggers and risk factors of HR in adults with mastocytosis in the ECNM registry.

Methods: Data were collected in 27 ECNM centers. We analyzed potential triggers (Hymenoptera venoms, food, drug, inhalant and others) and risk factors at diagnosis and during follow-up. The study group consisted of 2485 adults with mastocytosis, 1379 women (55.5%) and 1106 men (44.5%). Median age was 48.2 years (range 18-91 years).

Results: Nine hundred and forty eight patients (38.1%) reported one or more HR`. Most common triggers were Hymenoptera venoms in cutaneous mastocytosis (CM) and indolent systemic mastocytosis (ISM), whereas in advanced SM (advSM), most common elicitors were drugs, including nonsteroidal anti-inflammatory agents and penicillin. In multivariate analyses, tryptase level < 90 ng/mL, <15% infiltration by mast cells in bone marrow biopsy-sections, and diagnosis of ISM were identified as independent risk factors for HR. For drug-induced HR, prominent risk factors were advSM and high tryptase levels. New reactions were observed in 4.8% of all patients during 4 years follow-up.

Conclusions: HR are mainly triggered by Hymenoptera venoms in patients with CM and ISM and by drugs in patients with advSM. Tryptase levels <90 ng/mL, mast cell bone marrow infiltration <15%, and WHO category ISM are predictors of HR. New HR occur in 4.8% of all patients within 4 years.

Keywords: allergy; drug hypersensitivity; food hypersensitivity; hymenoptera venoms; immunohistochemistry; mastocytosis; tryptase.

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References

REFERENCES

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    1. Valent P, Horny HP, Escribano L, et al. Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leuk Res. 2001;25(7):603‐625.
    1. Valent P, Akin C, Sperr WR, et al. New insights into the pathogenesis of Mastocytosis: emerging concepts in diagnosis and therapy. Annu Rev Pathol. 2022;18:361‐386.
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    1. Valent P, Akin C, Hartmann K, et al. Updated diagnostic criteria and classification of mast cell disorders: a consensus proposal. HemaSphere. 2021;5(11):e646.

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