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冷球蛋白

Cryoproteins.

作者信息

Hobbs J R

出版信息

Ann Med Interne (Paris). 1986;137(3):254-9.

PMID:3767197
Abstract

Proteins producing phenomena after exposure to the cold are reviewed but concentrating mainly on those that can produce aggregates within the laboratory. From three large series the frequencies were I. Mixed Cryoglobulins (polyclonal 50 p. 100, monoclonal 18 p. 100); II. Monoclonal Cryoglobulin (25 p. 100); III. Cryofibrinogen (3 p. 100) and IV. Cold Agglutinins (polyclonal 2 p. 100, monoclonal 2 p. 100). Mixed cryoglobulins present with any of the features of immune complex disease, but with the onset of renal involvement, over 50 p. 100 of the patients are dead within 5 years. Monoclonal cryoglobulins present with flat lesions in dependent areas, most reflecting an underlying or impending malignant B-cell neoplasm. Cryofibrinogen, missed in heparinised samples, is associated with thrombophlebitis migrans and sometimes underlying cancer, especially of the pancreas. Cold agglutinins cause Raynaud's phenomenon and haemolytic anaemia and the blood must be centrifuged at 37 degrees C. Treatments are discussed and are certainly encouraging in about one-third of the patients.

摘要

本文综述了暴露于寒冷环境后会产生现象的蛋白质,但主要集中在那些能在实验室内形成聚集体的蛋白质。从三个大型系列研究中得出的频率如下:I. 混合性冷球蛋白(多克隆性占100分之50,单克隆性占100分之18);II. 单克隆冷球蛋白(占100分之25);III. 冷纤维蛋白原(占100分之3);IV. 冷凝集素(多克隆性占100分之2,单克隆性占100分之2)。混合性冷球蛋白具有免疫复合物疾病的任何特征,但随着肾脏受累的出现,超过100分之50的患者会在5年内死亡。单克隆冷球蛋白在下垂部位出现扁平病变,大多数反映潜在的或即将发生的恶性B细胞肿瘤。冷纤维蛋白原在肝素化样本中会被漏检,与游走性血栓性静脉炎有关,有时还与潜在的癌症有关,尤其是胰腺癌。冷凝集素会导致雷诺现象和溶血性贫血,血液必须在37摄氏度下离心。文中讨论了治疗方法,约三分之一的患者治疗效果肯定令人鼓舞。

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