Mosesson M W, Wautier J L, Amrani D L, Dervichian M, Cattan D
J Lab Clin Med. 1982 Apr;99(4):559-67.
Cryofibrinogenemia was found in 10 of 24 plasma samples (42%) from subjects with FMF. This precipitate was found during active disease as well as during intervals between crises. We found a higher incidence of cryofibrinogenemia in subjects with mild to moderately severe disease not being treated with colchicine (six of eight) as compared with colchicine-treated subjects who were in partial or complete clinical remission (four or 16; p less than 0.02). All cryofibrinogen precipitates contained fibrin, as assessed by electrophoretic analyses showing the presence of multimeric crosslinked forms of fibrin(ogen) linked by gamma-dimers. This finding in clinical specimens supports the hypothesis that fibrin in an obligatory component of cryofibrinogen. Fibrin was also found in HPF (two of six specimens) prepared from cryofibrinogen-negative FMF plasmas, thus showing that soluble forms of fibrin are even more prevalent in this disorder than is indicated by the frequent finding of cryofibrinogenemia.
在24例家族性地中海热(FMF)患者的血浆样本中,有10例(42%)检测到冷纤维蛋白原血症。这种沉淀物在疾病活动期以及发作间期均能被发现。我们发现,未接受秋水仙碱治疗的轻至中度严重疾病患者中,冷纤维蛋白原血症的发生率更高(8例中有6例),相比之下,处于部分或完全临床缓解期的接受秋水仙碱治疗的患者中,冷纤维蛋白原血症的发生率为4/16(p<0.02)。通过电泳分析评估,所有冷纤维蛋白原沉淀物均含有纤维蛋白,结果显示存在由γ-二聚体连接的多聚体交联形式的纤维蛋白(原)。临床标本中的这一发现支持了冷纤维蛋白原中纤维蛋白是必需成分的假说。在由冷纤维蛋白原阴性的FMF血浆制备的高倍视野(HPF)中也发现了纤维蛋白(6个标本中有2个),因此表明,在这种疾病中,可溶性纤维蛋白形式比冷纤维蛋白原血症的频繁发现所显示的更为普遍。