Why the 20% + 2 Tryptase Formula Is a Diagnostic Gold Standard for Severe Systemic Mast Cell Activation and Mast Cell Activation Syndrome

Valent, Peter; Bonadonna, Patrizia; Hartmann, Karin; Broesby‐Olsen, Sigurd; Brockow, Knut; Butterfield, Joseph H.; Triggiani, Massimo; Lyons, Jonathan J.; Elberink, J. N. G. Oude; Arock, Michel; Metcalfe, Dean D.; Akin, Cem

doi:10.1159/000501079

Cited by 98 publications

(95 citation statements)

References 56 publications

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“…2 A mast cell activation is very likely if tryptase measurement at the acute moment is higher than baseline level, namely 1.2 times baseline plus two, as confirmed in our case. 21 There is no evidence that chlorhexidine allergy can resolve over time. Life-time avoidance is indicated.…”

Section: Discussionmentioning

confidence: 99%

Anaphylaxis related to disinfection with chlorhexidine in a teenager treated for cancer

Devinck

Bauters

Lapeere

et al. 2020

J Oncol Pharm Pract

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Introduction Literature shows upcoming allergy to chlorhexidine due to the widespread use of the disinfectant within and outside surgical settings. Only a few case reports have been published regarding the use of topical chlorhexidine disinfectant outside surgery and only a minority of these within the pediatric population. Case report We present a case-report of a teenager, treated for acute lymphoblastic leukemia who developed an anaphylactic shock after repeated chlorhexidine use for skin disinfection at the insertion of a central venous catheter during his chemotherapy treatment. Preceding minor symptoms such as local swelling and pruritus were not recognized as possible allergy to chlorhexidine. Management and outcome: He was treated with two doses of intramuscular adrenaline and transferred to the pediatric intensive care unit where he fully recovered. Specific IgE testing was positive for chlorhexidine. A total avoidance of chlorhexidine was instructed. Discussion A similar case was published regarding an anaphylaxis after use of chlorhexidine disinfectant for a dialysis catheter. Almost all other case reports of anaphylactic shock were found within surgical settings or after insertion of an impregnated central venous catheter/urine catheter. We suggest that some of the disinfectant might have been flushed in the catheter and then caused an anaphylactic reaction. The link between symptoms and chlorhexidine was not made until an anaphylactic reaction occurred. Literature data show that chlorhexidine often causes mild preceding symptoms before an anaphylaxis occurs. So let awareness arise around this ‘hidden allergen’ of which warning reactions often are being missed.

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Section: Discussionmentioning

confidence: 99%

Anaphylaxis related to disinfection with chlorhexidine in a teenager treated for cancer

Devinck

Bauters

Lapeere

et al. 2020

J Oncol Pharm Pract

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“…It requires to be analyzed through a fluoroimmune enzyme assay and, ideally, the blood sample should be promptly collected after onset of symptoms because of the short half-life of the enzyme. In order to define a significative increase in tryptase levels above the baseline, the level of the enzyme should be higher than 20% plus 2 ng/mL [ 21 ]. When a baseline level prior to the onset of acute phase is not available, it can be detected at least 24 h following acute phase’s complete resolution.…”

Section: Definition Of Mcasmentioning

confidence: 99%

Mast Cell Activation Disorders

et al. 2021

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Background and Objectives: Mast cell disorders comprise a wide spectrum of syndromes caused by mast cells’ degranulation with acute or chronic clinical manifestations. Materials and Methods: In this review article we reviewed the latest findings in scientific papers about mast cell disorders with a particular focus on mast cell activation syndrome and mastocytosis in pediatric age. Results: Patients with mast cell activation syndrome have a normal number of mast cells that are hyperreactive upon stimulation of various triggers. We tried to emphasize the diagnostic criteria, differential diagnosis, and therapeutic strategies. Another primary mast cell disorder is mastocytosis, a condition with a long-known disease, in which patients have an increased number of mast cells that accumulate in different regions of the body with different clinical evolution in pediatric age. Conclusions: Mast cell activation syndrome overlaps with different clinical entities. No consensus was found on biomarkers and no clearly resolutive treatment is available. Therefore, a more detailed knowledge of this syndrome is of fundamental importance for a correct diagnosis and effective therapy.

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“…Serial measurement of tryptase levels during an anaphylactic episode, and measurement of a baseline level after recovery are reported to be more useful than measurement at only one point in time (85). The "20% + 2 formula" has been validated in clinical practice and currently considered significant in clinical practice as a criterion of severe systemic mast cell activation and mast cell activation syndrome (MCAS) (86). Histamine is also a marker of mast cell and basophil activation.…”

Section: Biomarkersmentioning

confidence: 99%

Anaphylaxis

Bilò

Martini

Tontini

et al. 2020

Eur Ann Allergy Clin Immunol

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Anaphylaxis is the most severe systemic hypersensitivity reaction, and it can be life-threatening or even fatal. It involves the activation of multiple immune and non immune pathways beyond IgE, thus exhibiting different phenotypes. New symptoms of hypersensitivity caused by chemotherapy drugs, monoclonal antibodies, and biological agents have been suggested to be recognized as anaphylaxis phenotypes. No biomarker has been described that allows an unequivocal diagnosis of anaphylaxis. Moreover, more biomarkers for specific endotypes are needed to stratify severity, to predict risk, and to optimaze tretament choice in the individual patient. Food, drugs and stinging insects represent the most commly identified triggers. Idiopathic anaphylaxis is a diagnosis of exclusion and it can hide a clonal mast cell disorder. Individual risk factors and co-factors may influence the severity of anaphylaxis or its onset, and they should be identified to implement the appropriate measures to prevent recurrence. Prompt recognition and treatment are critical in anaphylaxis, adrenaline being the first-line saving therapy. Individualized anaphylaxis action plan should include avoidance measures, prescription of an adrenaline autoinjector, education, optimal management of relevant comorbidities, venom specific immunotherapy, food oral immunotherapy, and drug desensitization, when appropriate. However, the quality of acute and long-term anaphylaxis management is variable influencing the poor outcomes experienced by many patients. Clinical practice guidelines have the potential to improve outcomes, but they often prove challenging to implement in routine clinical care.

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Why the 20% + 2 Tryptase Formula Is a Diagnostic Gold Standard for Severe Systemic Mast Cell Activation and Mast Cell Activation Syndrome

Cited by 98 publications

References 56 publications

Anaphylaxis related to disinfection with chlorhexidine in a teenager treated for cancer

Anaphylaxis related to disinfection with chlorhexidine in a teenager treated for cancer

Mast Cell Activation Disorders

Anaphylaxis

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