一个“健康”家庭中的早期补体成分缺乏与混合性冷球蛋白血症

Early complement component depletion and mixed cryoglobulinemia in a "healthy" family.

作者信息

Waterman J R, Winkelstein J A, Berzofsky R N, Hsu S H, Bias W B, Arnett F C

出版信息

Arthritis Rheum. 1979 Sep;22(9):1006-12. doi: 10.1002/art.1780220909.

DOI:10.1002/art.1780220909

Abstract

A family containing two apparently healthy brothers with profound early complement component depletion (C1, C4, C2) and mixed IgG-IgM polyclonal cryoglobulins was studied. The cryoglobulins possessed rheumatoid factor activity and depleted early complement components in normal human serum. Circulating immune complexes could not be detected by utilizing standard methods. The phenomenon was not HLA-linked. This study demonstrates the familial occurrence of a chronic hypocomplementemic state associated with cryoglobulinemia in clinically normal subjects.

摘要

对一个家族进行了研究，该家族中有两个明显健康的兄弟，他们存在严重的早期补体成分耗竭（C1、C4、C2）以及混合性IgG-IgM多克隆冷球蛋白。这些冷球蛋白具有类风湿因子活性，并能使正常人血清中的早期补体成分耗竭。利用标准方法无法检测到循环免疫复合物。这种现象与HLA无关。本研究证明了在临床正常的受试者中，与冷球蛋白血症相关的慢性低补体血症状态存在家族聚集性。