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通过血清肥大细胞类胰蛋白酶水平升高检测到的与严重黄蜂蜇伤过敏反应相关的肥大细胞增多症。

Mastocytosis associated with severe wasp sting anaphylaxis detected by elevated serum mast cell tryptase levels.

作者信息

Biedermann T, Ruëff F, Sander C A, Przybilla B

机构信息

Klinik und Poliklinik für Dermatologie und Allergologie, Ludwig-Maximillians-Universität München, Munich, Germany.

出版信息

Br J Dermatol. 1999 Dec;141(6):1110-2. doi: 10.1046/j.1365-2133.1999.03214.x.

Abstract

A markedly elevated serum level of mast cell tryptase (77.6 microg/L; 95th percentile in normals 13.5 microg/L) was detected in a patient treated for 5 years with wasp venom immunotherapy because of severe anaphylaxis following a wasp sting. Retrospective analysis of stored serum samples taken during the course of immunotherapy revealed that the tryptase level had been elevated for at least 3 years. Despite several dermatological examinations, skin changes of mastocytosis had been missed. Re-examination of the patient revealed sparse macules on the thorax and thighs; Darier's sign was negative. Histologically, mast cell accumulation in these lesions was demonstrable. No signs of systemic mastocytosis were detected. The most appropriate diagnosis was telangiectasia macularis eruptiva perstans. Even in patients with highly elevated tryptase levels, mastocytosis may go undiagnosed. As mastocytosis predisposes to severe anaphylaxis, the condition should be looked for in patients with such reactions by clinical examination and measurement of serum tryptase levels.

摘要

一名因黄蜂蜇伤后发生严重过敏反应而接受黄蜂毒液免疫治疗5年的患者,检测到其血清肥大细胞类胰蛋白酶水平显著升高(77.6μg/L;正常人群第95百分位数为13.5μg/L)。对免疫治疗过程中采集的储存血清样本进行回顾性分析发现,类胰蛋白酶水平升高至少已有3年。尽管进行了多次皮肤科检查,但肥大细胞增多症的皮肤改变仍被漏诊。对该患者再次检查发现胸部和大腿有散在的斑疹; Darier征阴性。组织学检查显示这些病变中有肥大细胞聚集。未检测到系统性肥大细胞增多症的迹象。最恰当的诊断是持久性斑疹性毛细血管扩张症。即使在类胰蛋白酶水平高度升高的患者中,肥大细胞增多症也可能未被诊断出来。由于肥大细胞增多症易导致严重过敏反应,对于有此类反应的患者,应通过临床检查和检测血清类胰蛋白酶水平来排查该病。

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