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. 2022 Mar;12(3):e12132.
doi: 10.1002/clt2.12132.

Mastocytosis presenting with mast cell-mediator release-associated symptoms elicited by cyclo oxygenase inhibitors: prevalence, clinical, and laboratory features

Tiago Azenha Rama  1   2 , José Mário Morgado  3   4 , Ana Henriques  3   4 , Luis Escribano  4   5 , Iván Alvarez-Twose  3   4 , Laura Sanchez-Muñoz  3   4 , André Moreira  1   2   6 , José Romão  7   8 , Alberto Órfão  4   5 , Almudena Matito  3   4
Affiliations
Free PMC article

Mastocytosis presenting with mast cell-mediator release-associated symptoms elicited by cyclo oxygenase inhibitors: prevalence, clinical, and laboratory features

Tiago Azenha Rama et al. Clin Transl Allergy. 2022 Mar.
Free PMC article

Abstract

Background: Nonsteroidal anti-inflammatory drugs (NSAIDs) are frequently avoided in mastocytosis, because of a potential increased risk for drug hypersensitivity reactions (DHRs) due to inhibition of cyclo-oxygenase (COX), subsequent depletion of prostaglandin E2 and release of leukotrienes.

Objectives: Here, we aimed at determining the prevalence of mast cell (MC) mediator release symptoms triggered by NSAIDs in mastocytosis patients and the associated clinical and laboratory features of the disease.

Methods: Medical records from 418 adults to 223 pediatric mastocytosis patients were retrospectively reviewed. Patients were classified according to tolerance patterns to NSAIDs and other COX inhibitors (COXi) and compared for epidemiological, clinical and laboratory findings.

Results: Overall, 87% of adults and 91% of pediatric patients tolerated NSAIDs and other COXi. Among adult and pediatric patients presenting DHRs, 5% and 0% reacted to multiple NSAIDs, 4% and 0.7% were single reactors, and 3% and 8% were single reactors with known tolerance to paracetamol but unknown tolerance to other COXi, respectively. Among adults, hypersensitivity to ≥2 drugs was more frequent among females (p = 0.009), patients with prior history of anaphylaxis to triggers other than NSAIDs or other COXi and Hymenoptera venom (p = 0.009), presence of baseline flushing (p = 0.02), baseline serum tryptase ≥48 ng/ml (p = 0.005) and multilineage KIT mutation (p = 0.02). In contrast, tolerance to NSAIDs and other COXi was more frequent among males (p = 0.02), in patients with anaphylaxis caused by Hymenoptera venom (p = 0.02), among individuals who had skin lesions due to mastocytosis (p = 0.01), and in cases that had no baseline pruritus (p = 0.006). Based on these parameters, a score model was designed to stratify mastocytosis patients who have never received NSAIDs or other COXi apart from paracetamol, according to their risk of DHR.

Conclusions: Our results suggest that despite the frequency of MC mediator related symptoms elicited by NSAIDs and other COXi apart from paracetamol is increased among mastocytosis patients versus the general population, it is lower than previously estimated and associated with unique disease features. Patients that tolerated NSAIDs and other COXi following disease onset should keep using them. In turn, adults with unknown tolerance to such drugs and a positive score should be challenged with a preferential/selective COX-2 inhibitor, while the remaining may be challenged with ibuprofen.

Keywords: anaphylaxis; mast cell-mediator release-associated symptoms; mast cells; mastocytosis; non-steroidal anti-inflammatory drug hypersensitivity.

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Conflict of interest statement

The authors have no conflicts of interest relevant to this article to disclose.

Figures

FIGURE 1
FIGURE 1
Score model proposed to screen for hypersensitivity to multiple non‐steroidal anti‐inflammatory drugs or other cyclo‐oxygenase inhibitors drugs, in adult patients with mastocytosis
FIGURE 2
FIGURE 2
Decision‐tree algorithm for adult mastocytosis patients at risk of drug hypersensitivity reaction and associated with the administration of non‐steroidal anti‐inflammatory drugs and other cyclo‐oxygenase inhibitors apart from paracetamol
See this image and copyright information in PMC

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References

    1. Akin C, Valent P. Diagnostic criteria and classification of mastocytosis in 2014. Immunol Allergy Clin North Am. 2014;34(2):207–218. - PubMed
    1. Valent P, Horny H, Li C, et al. Mastocytosis (Mast cell disease). In: Jaffe ES, Harris NL, Stein H, Vardiman JW, Eds. World Health Organization (WHO) Classification of Tumours Pathology & Genetics Tumours of Haematopoietic and Lymphoid Tissues. IARC Press; 2001:291–302.
    1. Cohen SS, Skovbo S, Vestergaard H, et al. Epidemiology of systemic mastocytosis in Denmark. Br J Haematol. 2014;166(4):521–528. - PubMed
    1. Doormaal JJ, Arends S, Brunekreeft KL, et al. Prevalence of indolent systemic mastocytosis in a Dutch region. J Allergy Clin Immunol. 2013;131(5):1429–1431e1. - PubMed
    1. Horny H‐P, Akin C, Arber DA, Peterson LC, Tefferi A, Metcalfe D, et al. Mastocytosis. In: Swerdlow S, Campo E, Harris N, Jaffe E, Pileri S , Stein H, et al., Eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Rev. 4th ed.). IARC; 2017.