Lipscombe T K, Orton D I, Bird A G, Wilkinson J D
Department of Dermatology, Amersham Hospital, United Kingdom.
Australas J Dermatol. 1996 Aug;37(3):145-8. doi: 10.1111/j.1440-0960.1996.tb01034.x.
Acquired C1-esterase inhibitor deficiency is a rare syndrome which usually presents with episodes of angioedema. Most patients have an underlying lymphoproliferative disorder. It is important to gastroenterologists, haematologists, immunologists and dermatologists, as these are the more likely specialties to which the patient will present. Recognition of the syndrome is important not only for diagnostic purposes but for investigating and treating any associated underlying lymphoproliferative disorders. In some instances the angioedema may precede the lymphoma by many years. Optimal patient management requires that both angioedema and the underlying lymphoma be recognized and treated.
获得性C1酯酶抑制剂缺乏症是一种罕见的综合征，通常表现为血管性水肿发作。大多数患者患有潜在的淋巴增殖性疾病。这对胃肠病学家、血液学家、免疫学家和皮肤科医生来说很重要，因为患者更有可能就诊于这些专科。认识该综合征不仅对诊断有重要意义，对调查和治疗任何相关的潜在淋巴增殖性疾病也很重要。在某些情况下，血管性水肿可能比淋巴瘤早出现许多年。最佳的患者管理要求同时识别和治疗血管性水肿及潜在的淋巴瘤。
