Nonaggressive systemic mastocytosis (SM) without skin lesions associated with insect-induced anaphylaxis shows unique features versus other indolent SM.

Abstract

BACKGROUND:

Indolent systemic mastocytosis (ISM) without skin lesions (ISMs(-)) shows a higher prevalence in males, lower serum baseline tryptase levels, and KIT mutation more frequently restricted to bone marrow (BM) mast cells (MCs) than ISM with skin lesions (ISMs(+)). Interestingly, in almost one-half of ISMs(-) patients, MC-mediator release episodes are triggered exclusively by insects.

OBJECTIVE:

We aimed to determine the clinical and laboratory features of ISMs(-) associated with insect-induced anaphylaxis (insectISMs(-)) versus other patients with ISM.

METHODS:

A total of 335 patients presenting with MC activation syndrome, including 143 insectISMs(-), 72 ISMs(-) triggered by other factors (otherISMs(-)), 56 ISMs(+), and 64 nonclonal MC activation syndrome, were studied.

RESULTS:

Compared with otherISMs(-) and ISMs(+) patients, insectISMs(-) cases showed marked male predominance (78% vs 53% and 46%; P < .001), a distinct pattern of MC-related symptoms, and significantly lower median serum baseline tryptase levels (22.4 vs 28.7 and 45.8 μg/L; P ≤ .009). Moreover, insectISMs(-) less frequently presented BM MC aggregates (46% vs 70% and 81%; P ≤ .001), and they systematically showed MC-restricted KIT mutation.

CONCLUSIONS:

ISMs(-) patients with anaphylaxis triggered exclusively by insects display clinical and laboratory features that are significantly different from other ISM cases, including other ISMs(-) and ISMs(+) patients, suggesting that they represent a unique subgroup of ISM with a particularly low BM MC burden in the absence of adverse prognostic factors.

• Authors: Iván Alvarez-Twose, Roberta Zanotti, David González-de-Olano, Patrizia Bonadonna, Arantza Vega, Almudena Matito, Laura Sánchez-Muñoz, José Mário Morgado, Omar Perbellini, Andrés García-Montero, Giovanna De Matteis, Cristina Teodósio, Maurizio Rossini, María Jara-Acevedo, Donatella Schena, Andrea Mayado, Alberto Zamò, Manuela Mollejo, Paula Sánchez-López, Nieves Cabañes, Alberto Orfao, Luis Escribano, Spanish Network on Mastocytosis (REMA), Italian Network on Mastocytosis (RIMA)
• Journal: The Journal of allergy and clinical immunology (J Allergy Clin Immunol) Vol. 133 Issue 2 Pg. 520-8 (Feb 2014) ISSN: 1097-6825 [Electronic] United States
• PMID: 23921094 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2013 American Academy of Allergy, Asthma & Immunology. Published by Mosby, Inc. All rights reserved.

Chemical References

• Allergens
• Immunoglobulin E
• Tryptases

Topics

• Adolescent
• Adult
• Aged
• Aged, 80 and over
• Allergens (immunology)
• Anaphylaxis (diagnosis, immunology)
• Animals
• Bees (immunology)
• Female
• Humans
• Immunoglobulin E (blood)
• Insect Bites and Stings (diagnosis, immunology)
• Male
• Mastocytosis, Systemic (diagnosis, immunology)
• Middle Aged
• Skin Diseases (diagnosis, immunology)
• Skin Tests
• Tryptases (blood)
• Wasps (immunology)
• Young Adult