Systemic mastocytosis presenting as intraoperative anaphylaxis with atypical features: a report of two cases
Systemic mastocytosis presenting as intraoperative anaphylaxis with atypical features: a report of two cases
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DOI：
10.1177/0310057x1304100120
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发表时间：
2013-01-01
影响因子：
1.5
通讯作者：
Platt, P. R.
中科院分区：
文献类型：
--
作者：
Bridgman, D. E.;Clarke, R. C.;Platt, P. R.
两例围手术期心血管崩溃事件发生后不久，肥大细胞类胰蛋白酶水平显著升高，导致假设免疫球蛋白E介导的药物诱导的过敏反应发生。然而，这两种情况下的临床表现是不典型的，随后的皮肤试验未能确定触发药物。几周后，进一步的血液检查显示肥大细胞类胰蛋白酶的基线水平持续升高。在这两种情况下，骨髓活检和基因检测证实了肥大细胞增多症的诊断。我们提供证据并进行推测。第一个案例中，松开止血带触发了肥大细胞脱粒，第二个案例中，暴露在花生中触发了肥大细胞脱粒。过敏反应的非典型表现应提醒麻醉师注意以前未诊断的肥大细胞增多症的可能性。
Two cases of perioperative cardiovascular collapse are presented that were associated with markedly elevated mast cell tryptase levels shortly after the event, leading to the assumption that an immunoglobin E-mediated, drug-induced anaphylaxis had occurred. However, the clinical picture in both cases was atypical and subsequent skin testing failed to identify a triggering drug. Further blood tests, some weeks later, revealed persistently elevated baseline levels of mast cell tryptase. In both cases bone marrow biopsy and genetic testing confirmed the diagnosis of mastocytosis. We present evidence and speculate. that mast cell degranulation was triggered by tourniquet release in the first case and by exposure to peanuts in the second. An atypical presentation of anaphylaxis should alert the anaesthetist to the possibility of previously undiagnosed mastocytosis.