Benitah E, Wautier J L, Cohen F, Herman D
Policlinique Médicale et Allergologie, Hôpital Lariboisière, Paris.
Ann Med Interne (Paris). 1988;139(7):488-90.
Acquired deficiency in C1 esterase inhibitor (C1 INH) was first described by Caldwell in 1972. Since that date, about 30 cases have been reported, in most cases during proliferative lymphocyte B syndromes. The acquired C1 INH deficiency can provoke episodes of angioneurotic edema as in hereditary AE. The complement profile differs, notably by the usual sudden fall in C1. Documented data suggest consumption of complement and therefore of C1 INH. The present case is the first reported of an acquired C1 INH deficiency during a cold hemagglutinin disease. The activation of complement by the classical pathway appears provoked by tumoral cells rather than a humoral factor, as suggested by the efficacy of anti-tumoral therapy in contrast to plasmapheresis in the present case. A possible mechanism for the C1 INH deficiency is discussed.
1972年，考德威尔首次描述了获得性C1酯酶抑制剂（C1 INH）缺乏症。自那时以来，已报告了约30例病例，大多数病例发生在增殖性淋巴细胞B综合征期间。获得性C1 INH缺乏症可引发与遗传性血管性水肿相同的血管性水肿发作。补体谱有所不同，尤其是C1通常会突然下降。有记录的数据表明补体以及C1 INH被消耗。本病例是首例报告的在冷凝集素病期间出现的获得性C1 INH缺乏症。与本病例中的血浆置换相反，抗肿瘤治疗的疗效表明，经典途径激活补体似乎是由肿瘤细胞而非体液因子引发的。本文讨论了C1 INH缺乏症的一种可能机制。
