Nonaggressive systemic mastocytosis (SM) without skin lesions associated with insect-induced anaphylaxis shows unique features versus other indolent SM.
J Allergy Clin Immunol
; 133(2): 520-8, 2014 Feb.
Article
em En
| MEDLINE
| ID: mdl-23921094
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.Palavras-chave
BM; Bone marrow; CLMast; Hymenoptera; ISM; ISMs(+); ISMs(−); Indolent systemic mastocytosis; Indolent systemic mastocytosis with skin lesions; Indolent systemic mastocytosis without skin lesions; Instituto de Estudios de Mastocitosis de Castilla La Mancha, The Clinical Reference Centre of the Spanish Network on Mastocytosis (REMA); MC; MCAS; Mast cell; Mast cell activation syndrome; REMA; SM; Serum baseline tryptase; Spanish Network on Mastocytosis; Systemic mastocytosis; VIT; Venom immunotherapy; WHO; World Health Organization; anaphylaxis; insect; insect sting anaphylaxis; mastocytosis; sBT
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Dermatopatias
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Vespas
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Abelhas
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Mastocitose Sistêmica
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Anafilaxia
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Mordeduras e Picadas de Insetos
Idioma:
En
Ano de publicação:
2014
Tipo de documento:
Article