Dacosta A, Guy J M, Cathebras P, Perrot J L, Decousus H, Tardy B, Gonthier R, Lamaud M, Rousset H, Verneyre H
Service de cardiologie, Hôpital Nord, Saint-Priest-en-Jarez.
Arch Mal Coeur Vaiss. 1993 Dec;86(12):1747-52.
The authors report three cases of syncope due to systemic mastocytosis. This is a rare cause of syncope but should be recalled in certain circumstances. In the light of these cases, the authors review the literature with respect to this unusual presentation. Syncope may occur at any age. Loss of consciousness may be more or less complete, brief or prolonged, isolated or recurrent and usually accompanied by prodromal symptoms. The least controversial physiopathogenic mechanism of these syncopes is intense vasoplegia induced by the release of vasoactive mediators, especially histamine. When there is a clinical suspicion of mastocytosis, even in the absence of skin changes, the diagnosis is confirmed by biopsy which shows abnormally high numbers of mastocytes irrespective of the organ biopsied. The treatment of acute forms with collapse is based on intravenous infusion of macromolecular fluids and injections of epinephrine. Prevention is by drugs which inhibit the synthesis of histamine, the degranulation of mastocytes and the production of prostaglandin D2.
作者报告了3例因系统性肥大细胞增多症导致的晕厥病例。这是晕厥的一种罕见病因,但在某些情况下应予以考虑。鉴于这些病例,作者回顾了关于这种不寻常表现的文献。晕厥可发生于任何年龄。意识丧失可能或多或少完全,短暂或持久,孤立或反复发作,且通常伴有前驱症状。这些晕厥最无争议的病理生理机制是血管活性介质尤其是组胺释放所诱发的强烈血管麻痹。当临床上怀疑有肥大细胞增多症时,即使没有皮肤改变,通过活检可确诊,活检显示无论活检器官如何,肥大细胞数量异常增多。急性虚脱型的治疗基于静脉输注大分子液体和注射肾上腺素。预防措施是使用抑制组胺合成、肥大细胞脱颗粒和前列腺素D2产生的药物。