获得性C1抑制剂缺乏症合并淋巴瘤导致复发性血管性水肿。
Acquired C1 inhibitor deficiency with lymphoma causing recurrent angioedema.
作者信息
Mathur R, Toghill P J, Johnston I D
机构信息
University Hospital, Nottingham, UK.
出版信息
Postgrad Med J. 1993 Aug;69(814):646-8. doi: 10.1136/pgmj.69.814.646.
Abstract
A 52 year old man who developed recurrent, massive and generalized angioedema for the first time during adult life was found to have an acquired deficiency of C1q esterase inhibitor (C1 INH) in association with a B cell lymphoma producing a paraprotein. He had low levels of C4 and C1 INH during the attacks which returned to normal after the successful treatment of lymphoma. An underlying lymphoproliferative disease should always be considered in adult patients with this immunological profile, recurrent angioedema and a negative family history.
摘要
一名52岁男性在成年后首次出现反复发作的、全身性的严重血管性水肿，发现其患有获得性C1q酯酶抑制剂（C1 INH）缺乏症，并伴有产生副蛋白的B细胞淋巴瘤。发作期间他的C4和C1 INH水平较低，淋巴瘤成功治疗后恢复正常。对于具有这种免疫特征、反复发作血管性水肿且家族史阴性的成年患者，应始终考虑潜在的淋巴增殖性疾病。
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