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

Indolent systemic mastocytosis (ISM) without skin lesions (ISMs鈭?/sup>) 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鈭?/sup> patients, MC-mediator release episodes are triggered exclusively by insects.

Objective

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

Methods

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

Results

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

Conclusions

ISMs鈭?/sup> patients with anaphylaxis triggered exclusively by insects display clinical and laboratory features that are significantly different from other ISM cases, including other ISMs鈭?/sup> 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.

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