Kors J W, van Doormaal J J, de Monchy J G
Department of Endocrinology, University Hospital, Groningen, The Netherlands.
J Intern Med. 1993 Mar;233(3):255-8. doi: 10.1111/j.1365-2796.1993.tb00984.x.
Systemic mastocytosis is a rare and chronic disorder characterized by a pathologically increased number of mast cells in various tissues and overproduction of mast cell mediators. From a group of 15 patients (10 females, 5 males) with systemic mastocytosis five female patients presented with a history of an anaphylactoid shock reaction to wasp sting. Three of them had no demonstrable specific IgE against wasp or bee venom in serum, and a skin test that was only weakly positive for wasp venom. One patient had specific IgE against wasp venom and a clearly positive skin test to wasp venom. The other patient had specific IgE against both wasp and bee venom and a skin test that was only weakly positive to wasp venom. Two patients had to stop a hyposensitization procedure because of systemic side effects. The five patients did not differ from the other patients with systemic mastocytosis with regard to either clinical symptoms and signs or urinary excretion of histamine metabolites. From the latter group two female and three male patients said they had been stung by a wasp in the past. Thus, anaphylactoid shock after Hymenoptera sting can be a presenting symptom of systemic mastocytosis and may be caused by an IgE- as well as a non-IgE-mediated mechanism. In cases of anaphylactoid reaction to Hymenoptera sting, especially when there is no IgE demonstrable in serum or in cases of intolerance of hyposensitization, the diagnosis of systemic mastocytosis should be considered, also in the absence of the clinical hallmarks of urticaria pigmentosa.
系统性肥大细胞增多症是一种罕见的慢性疾病，其特征是各种组织中肥大细胞数量在病理上增加以及肥大细胞介质过度产生。在一组15例系统性肥大细胞增多症患者（10名女性，5名男性）中，有5名女性患者有对黄蜂蜇伤发生类过敏休克反应的病史。其中3人血清中未检测到针对黄蜂或蜂毒的特异性IgE，皮肤试验对黄蜂毒液仅呈弱阳性。1名患者有针对黄蜂毒液的特异性IgE，对黄蜂毒液的皮肤试验呈明显阳性。另1名患者有针对黄蜂和蜂毒的特异性IgE，对黄蜂毒液的皮肤试验仅呈弱阳性。2名患者因全身副作用不得不停止脱敏治疗。这5名患者在临床症状和体征或组胺代谢产物的尿排泄方面与其他系统性肥大细胞增多症患者没有差异。后一组中有2名女性和3名男性患者表示他们过去曾被黄蜂蜇伤。因此，膜翅目昆虫蜇伤后的类过敏休克可能是系统性肥大细胞增多症的一个表现症状，可能由IgE介导以及非IgE介导的机制引起。在对膜翅目昆虫蜇伤发生类过敏反应的病例中，尤其是血清中未检测到IgE或对脱敏治疗不耐受的情况下，即使没有色素性荨麻疹的临床特征，也应考虑系统性肥大细胞增多症的诊断。
